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MINISTRY OF HEALTH OF UKRAINE UKRAINIAN MEDICAL STOMATOLOGICAL ACADEMY

Approved at the meeting of department of Pediatrics №2 Minutes № 21 " 05 " 06 2020 Head of the department MD, Prof. Kryuchko T.O. ______

METHODICAL RECOMMENDATIONS INDIVIDUAL WORK OF STUDENTS IN PREPARATION FOR THE PRACTICAL (SEMINARS) CLASS

Academic discipline Pediatrics Module № 1 The most common somatic diseases in children Topic of the lesson Functional gastrointestinal disorder in children of the early age. Course 4 Faculty Medical

Poltava-2020

1. Relevance of the topic: The question of functional disorders of the digestive system in pediatric gastroenterology is now one of the most pressing. Most often, parents consult a pediatrician for localized in the umbilical region, , poor appetite or refusal to eat. These complaints can occur in both functional and organic lesions of the digestive tract. Functional disorders are often found in the structure of pathology of the digestive system. According to a number of researchers, more than a third of patients with complaints of diseases of the digestive system, no organic disorders are detected. Namely, abdominal pain is functional in 90-95% of children, in 20% of cases in children is also due to functional disorders.

2. Specific objectives: - To analyze the etiological and pathogenetic factors of the most common functional gastrointestinal disorders in children: cyclic syndrome, colic, functional dyspepsia, functional . -Explain the principles of treatment, rehabilitation and prevention of functional gastrointestinal disorders in children: cyclic vomiting syndrome, colic, functional dyspepsia and functional constipation in young children. -To offer a differential diagnosis and make a preliminary diagnosis of cyclic vomiting, functional dyspepsia, colic and functional constipation in young children. - To classify and analyze the typical clinical picture of the most common functional gastrointestinal disorders in children: cyclic vomiting syndrome, colic, functional dyspepsia, functional constipation. -Treat to analyze the data of laboratory and instrumental examinations in the typical course of the most common functional gastrointestinal disorders in children: general clinical and urine tests, biochemical blood tests, coprogram, study of intestinal microflora, ultrasound of internal organs, fibroesophageal gastroduodenum. -Analyze the prognosis of life in functional gastrointestinal disorders in children: the syndrome of cyclic vomiting, colic, functional dyspepsia and functional constipation in young children. - Diagnose and provide emergency care for colic in young children.

3. Basic knowledge, skills, abilities necessary for studying the topic (interdisciplinary integration): № Names of previous Acquired skills disciplines 1. Normal anatomy Know the anatomical features of the in children 2. Normal physiology Have an idea of the physiological processes that occur in the digestive tract in children. 3. Pathological physiology Identify pathophysiological processes that occur in the gastrointestinal tract on the basis of symptoms 4. Microbiology with virology Have knowledge of the normal composition of the intestinal and immunology microflora of newborns and young children. 5. Patient care and nursing Demonstrate skills in caring for young children with functional practice disorders of the gastrointestinal tract and providing pre-medical care. 6. Propaedeutic pediatrics To have knowledge about the main clinical symptoms of functional gastrointestinal disorders in young children, to have an idea of laboratory and instrumental methods of examination. 7. Social medicine and health Apply knowledge about the structure of health care for children care organization for the proper use of resources of the health care system in terms of treatment and prevention of functional disorders of the gastrointestinal tract. 8. Have knowledge of the main groups of used in the treatment of functional gastrointestinal disorders in young children.

4. Tasks for independent work in preparation for the lesson. 4.1. The list of the basic terms, parameters, characteristics which the student should master at preparation for employment № Term Definition 1. Functional gastrointestinal It is a diverse combination of gastrointestinal symptoms disorders without structural or biochemical abnormalities. 2. Cyclic vomiting syndrome The disease is predominantly childhood, manifested by (CVS) stereotyped recurrent episodes of vomiting, followed by symptoms of complete well-being. 3. Functional dyspepsia according to Roman criteria III is defined as the presence of signs of dyspepsia originating from the gastroduodenal region in the absence of any organic, systemic or metabolic diseases that could explain these signs. 4. Functional constipation Delayed bowel movements, which is accompanied by difficulty in in the absence of organic pathology.

4.2. Theoretical questions for the lesson: 1. Define the concepts: functional gastrointestinal disorders, cyclic vomiting syndrome, functional constipation, functional dyspepsia. 2. Causes of functional gastrointestinal disorders in children. 3. Classification of functional gastrointestinal disorders in children according to Roman criteria III-IV. 4. Pathogenesis of functional gastrointestinal disorders in children. 5. Clinic of cyclic vomiting syndrome. 6. Criteria for the diagnosis of cyclic vomiting syndrome. 7. Diagnostic criteria for functional dyspepsia in children. 8. The main symptoms of functional dyspepsia in children. 9. Mechanisms of symptoms of functional dyspepsia. 10. Clinic and diagnosis of colic in young children. 11. Clinic and diagnosis of functional constipation in young children. 12. Treatment, prevention and prognosis of functional gastrointestinal disorders in young children.

4.3. Practical work (tasks) performed in class: 1. Work with test tasks. 2. Work of students in the wards at the bedside of children with functional gastrointestinal disorders: A) Collection of complaints, medical history; B) Objective examination of children; C) Formulation of the diagnosis according to the modern classification; D) Make a plan for the examination of a sick child; E) Make a treatment plan for a sick child. 3. Clinical analysis of an indicative case. 4. Solving situational tasks.

5. Contents of the topic:

Definition:Functional gastrointestinal disorders are disorders of the interaction between the intestine and the brain (Drossman, 2016). This group of diseases is associated with any combination of disorders of the following gastrointestinal functions: motility, visceral hypersensitivity, mucosal disorders and its immune functions (mucosal homeostasis), changes in the composition of the intestinal microbiota, combined with changes in the central (CNS). Etiopathogenesis: today formed a new integrated biopsychosocial model of development of functional gastrointestinal disorders, which explains the development of these disorders as a result of interaction of biological, psychological and social subsystems at different levels and determine the degree of manifestation of various symptoms in gastrointestinal tract (functional disorders of the gastrointestinal tract). and those around her. The biopsychosocial model considers some infectious and non-infectious factors that are part of these subsystems as comorbid. At an early age, genetics, sociocultural environmental factors can negatively affect psychosocial development in terms of personal qualities, propensity to life stress, psychological state, as well as cognition and coping skills. These factors also affect the degree of sensitivity to intestinal dysfunction: altered motility or sensitivity, altered immune dysfunction of the mucosa or , a certain microbial environment and the influx of food and nutrients. In addition, all factors of connection between the brain and intestines interact with the degree of excitability of the CNS. Although psychosocial factors are not required for diagnosis, they affect the physiological functioning of the gastrointestinal tract through the axis "brain - intestine" (motility, sensitivity, barrier function) and are modulators of pain and symptoms of patient behavior, and ultimately affect treatment choices and clinical results.

Classification. The classification of functional gastrointestinal disorders in infants and children is based on the age of the child (taking into account certain anatomical and physiological features of infants and the symptom that predominates in the complaints with which patients seek medical attention. Classification of functional gastrointestinal disorders in infants and children (Roman diagnostic criteria IV) Pediatric functional gastrointestinal disorders: Newborns / young children G1. Infant Regurgitation or pediatric regurgitation G2. Infant Rumination Syndrome G3. Cyclic Vomiting Syndrome G4. Colic in infants (or - InfantColic) G5. Functional diarrhea G6. InfantDyschezia G7. Functional constipation Children's functional gastrointestinal disorders (FGD): Children / Adolescents H1. Disorders accompanied by and vomiting H1a. Cyclic vomit syndrome (CVS) H1b. Functional nausea and functional vomiting H1c. Rumination syndrome H1d.Aerophagia H2. Functional abdominal pain disorders H2a. Functional dyspepsia · H2a1 Postpranidal distress syndrome · H2a2 Epigastric pain syndrome H2b. H2c. Abdominal migraine H2d.Functional abdominal pain not otherwise specified H3 Functional defecation disorders H3a.Functional constipation H3b. Non retentive fecalin continence

Symptoms of FGR do not disrupt the normal development of the child, are not associated with acute , are not complicated, have no signs of chronic gastrointestinal disease and should last at least 1-3 months before the diagnosis of functional gastrointestinal disorders. symptoms (red flags): • pain during the act of defecation, • , • vomiting coffee grounds, • bleeding, • manifestations of anemia, • weight loss • a sharp decrease or complete lack of appetite, • increase in body temperature In the presence of these symptoms, the functionality of the disease is unlikely and additional instrumental and laboratory examinations are mandatory at the initial visit of parents to the doctor.

REGURGITATION IN CHILDREN -is a passive dumping of gastric contents into the pharynx and oral cavity, which can be seen from the outside.

Diagnostic criteria of Regurgitation, Roman criteria IV The diagnosis is established in the presence of all the following symptoms in healthy infants from 3 weeks to 12 months of age: · Vomiting 2 or more times a day for 3 or more weeks; · Lack of vomiting, bleeding, aspiration, , difficulty swallowing, feeding, forced position, painful belching, insufficient body weight gain The presence of any of these symptoms can be considered as GERD

Differential diagnosis: 1. Congenital malformations of the oropharynx, chest, lungs, central nervous system, heart and gastrointestinal tract 2. GERD (gastroesophageal reflux disease) 3. Diseases not related to the gastrointestinal tract (endocrine, metabolic, enzymopathy)

Treatment includes three main approaches: 1. Restoration of emotional comfort in the family, the correct mode of the day and feeding regime, according to age. 2. Prevention of vomiting by ensuring the appropriate position of the body (postural therapy) after feeding, and compliance with the rules of bottle feeding during artificial feeding; 3. Alimentary correction; According to the latest data, therapy does not give positive results and sometimes worsens the child's condition

Rumination Syndrome It is characterized by periodic attacks of contractions of the abdominal muscles, diaphragm and tongue, leading to the dumping of gastric contents into the oral cavity, where it is re- chewed and swallowed or removed from the mouth. Diagnostic criteria of Rumination, Roman criteria IV The diagnosis is established in the presence of signs within, 2 months: 1. Repeated contractions of the abdominal muscles, diaphragm and tongue; 2. Regurgitation (throwing) of gastric contents into the oral cavity, which is either coughed up or chewed and re-slipped 3. 3 of the following criteria must be present: a) begins shortly after a meal at the age of 3-8 months; b) does not occur during sleep and communication with others; c) does not respond to standard treatment used for gastroesophageal reflux. d) is not accompanied by violations of the general condition

Treatment: 1. Satisfaction of physical and emotional needs of the baby. (Unfortunately, in mentally retarded children, adequate care may not be sufficient for the treatment of rumination syndrome) 2. Behavioral therapy, with the involvement of positive strengthening and methods that create disgust 3. Rational . In older children and adolescents is the method of choice for the treatment of rumination 4. Changing the composition of food, slower absorption of food and limiting fluid intake during meals

AEROPHAGY -Feeling of distension in the epigastrium, which occurs due to excessive ingestion of air and decreases after belching air. Common factors that contribute to the symptoms of aerophagia: 1. Various psychogenic disorders (stress, nervous shock, etc.) and hysterical disorders 2. Respiratory diseases due to difficulty , resulting in the oral mucosa dries out and the child is forced to swallow often; 3. Hasty eating, insufficiently chewed food; 4. Insufficiency of the NSS (cardia), of the esophageal orifice of the diaphragm.

Diagnostic criteria of Aerophagia, Roman criteria IV The diagnosis is established in the presence of at least 2 signs listed below: • Ingestion of air. • due to accumulation of air in the intestines. • Recurrent belching and / or increased flatulence. These symptoms are observed at least once a week for 2 months before diagnosis.

Treatment: 1. Information impact, consultation with a psychologist 2. Dietary recommendations (refusal of lollipops, chewing gum and carbonated drinks, slow eating in small sips); 3. At their inefficiency are shown: (tranquilizers), with easy neuroleptic effect (etaperazine, thietylperazine)

CYCLIC VOMITING SYNDROME (SCB) disease mainly in childhood, which is manifested by stereotyped recurrent episodes of vomiting, followed by periods of complete well-being. Etiopathogenetic mechanisms of SCB development: Currently, views are expressed on SCB as a polyetiological disease, which is based on disorders of the hypothalamic-pituitary-adrenal system (increased secretion of corticotropin-releasing factor andvegetative regulation) (sympathicotonia). It is established that stressful activation of the hypothalamic-hypothalamic system . Previous studies have shown that sympathetic hyperactivity and autonomic dysfunction make a significant contribution to the pathogenesis of STDs. In particular, such signs of STDs as pallor, hyperemia, orthostatic , fever, lethargy, salivation and diarrhea are closely related to dysfunction of the autonomic nervous system. In a number of studies, evidence of dysfunction of the ANS in SCB. SCB is also considered a mitochondrial disease and ionic membrane canalopathy, which leads to increased excitability of neurons. Very often in the literature the association with migraine is carried out that is reflected in additional diagnostic criteria. It is known that the central and peripheral effects of the trigeminal-vascular system are largely realized and modulated through serotonin receptors. Among the variety of serotonin receptors for migraine are 5-HT1, 5-HT2 and 5-HT3 receptors. In this case, 5-HT3 receptors are also related to the occurrence of nausea and vomiting, which in migraine often occur due to stimulation of these receptors, which are located mainly in the centers of nausea and vomiting of the lower parts of the brainstem. detection of migraine in relatives of patients with CVS. Considering, Provoking factors: • infection (41%); • psychological stress (34%); • foods, including histamine serotonin-containing; • physical exhaustion or lack of sleep (18%); • atopic reactions (13%); • menstruation (13%) and other factors. Clinic.In the clinical picture of SCB there are four periods: interictal, prodromal, seizure period and recovery period (Fig. 2).

In the interictal period, children feel good and often do not even want to talk or even think about the suffering they endured while they were sick.

Prodromal periodThis is the period of time during which the patient begins to feel the approach of an episode of cyclic vomiting, but can still prevent it by taking by mouth. This period ends with the onset of vomiting. The prodromal period can last from a few minutes to several hours and even several days. However, this period is often absent. Thus, the symptoms of STDs are found in only 25% of children in the prodromal period. However, due to the uniformity of the onset of the disease, patients mostly experience an approaching attack. This feature of the SCB should be used for preventive measures.

Paroxysmal periodcharacterized by uncontrollable nausea and vomiting, which are observed in all (100%) children. Vomiting can occur up to 6 times in an hour and can be mixed with bile (76%) and blood (32%). This makes it impossible to drink or eat any food or medicine. Symptoms that may accompany STDs: paleness, weakness, salivation, abdominal pain, hypersensitivity to sounds, odors and light, headache, frequent loose stools, fever, tachycardia, hypertension, skin spots and leukocytosis.

During the recovery period there is an increase in the activity of the child, restoration of appetite, normalization of skin color, positive emotions return, water-salt balance is gradually restored

Diagnostic criteria of the International Association of SCB, Roman criteriaIV All symptoms should be present and other pathology excluded, which may be accompanied by periodic vomiting 1. Two or more episodes of paroxysmal persistent vomiting with or without urge to vomit for several hours or days for 6 months. 2. Stereotypes for each patient 3. Between episodes of vomiting for weeks or months, the child feels satisfactory. Returns to baseline health Additional criteria: -stereotype: for a particular patient, each episode is similar in timebeginning, intensity, duration, frequency (either sporadic or with a certaininterval), associated ; - possibility of self-elimination (attacks can come to an end spontaneously and without treatment); -anamnesis (cases of migraine or STD in the family, often through the mother); If the child has all the main symptoms of STDs, the diagnosis can be considered established, but, however, even in this case, additional paraclinical studies and a thorough differential diagnosis are required. Differential diagnostics: with neurological, urological, metabolic, endocrine pathology and gastrointestinal malformations in children if symptoms began before the age of 3 years. -older children should also exclude inflammatory diseases of the upper digestive tract. Mandatory minimum examinations for repeated vomiting of the child before infusion therapy: , glucose, nitrogen urea, creatinine (amino acids, ACTH natriuretic hormone, level 17 oxyketosteroids, organic acids in suspected endocrine and metabolic diseases). The results of timely laboratory tests may reveal violations of fatty acid oxidation or partial deficiency of ornithine transcarbamylase. The strategy of drug treatment is based on the fact that treatment depends on the period of the disease. For each period there is a purpose and treatment options that allow a comprehensive approach to the treatment of STDs

Therapeutic tactics for cyclic vomiting syndrome in children Periods I II III IV Symptoms Missing Prodroma Attack Recovery The purpose of Attack Seizure abortion Termination of the Restoration of therapy prevention attack and if food without unsuccessful, recurrence sedation until the end of the attack Therapy Elimination of Lozepam and / or Intravenous fluid Extend the diet if triggers. ondansetron (peros replenishment in it is tolerated Migraine or sublingually) combination with prevention Sleep H2-blockers. For (cyproheptadine, Analgesia (for the term of the aminotriptyline, abdominal pain). attack intravenous propranolol) Triptan (for lorazepam or headache) ondasetron. For sedation chlorpromazine (0.5-1.0 mg / kg) and diphenylhydramine (0.5-1.0 mg / kg) Repeat every 3-4 hours for nausea in the waking state

Functional dyspepsia - symptom complex manifested in children older than 1 year (usually after 2 years), when the child may complain of pain , and partially distinguish the upper and lower abdomen

Diagnostic criteria for functional dyspepsia, Roman criteria IV The diagnosis is established in the presence of all the signs listed below: a) Constant or recurrent pain or discomfort (feeling of early satiety, nausea) in the upper abdomen (above the navel), local swelling of the epigastrium. b) Pain does not decrease after defecation or is not associated with a change in frequency or the nature of the stool. c) There is no evidence of an inflammatory, metabolic or neoplastic process, as well anatomical changes of the canal, which can explain the revealed signs of the disease. These symptoms are observed at least once a week for, at least 2 months before diagnosis. Clinically, there are two options for the course of this disorder: postpranide distress syndrome (heaviness and discomfort after eating) or with epigastric pain syndrome, depending on what symptoms predominate in the clinic.

Differential diagnosis is performed with inflammatory diseases of the upper part digestive tract (gastritis, gastroduodenitis. gastric ulcer and duodenal ulcer, helminthiasis, diseases of the gallbladder and pancreas, malformations digestive tract and pancreas)

Treatment of functional dyspepsia: 1. Mandatory component of the therapy of functional dyspepsia is the normalization of the autonomic status and psycho-emotional state, consultation with a psychoneurologist, psychologist. 2. Diet in functional dyspepsia is largely determined by the individual tolerability of products. Also excluded are all foods that can cause pain epigastrium, , belching: fatty foods, smoked sausages, strong meat, fish and mushrooms broths, soups, borscht, rye bread, fresh pastries, pancakes, carbonated drinks, coffee, radishes, spicy condiments. Children are allowed to eat white bread, better yesterday, crackers with white bread, biscuits, vegetarian soups and soups in weak broths, soup, boiled meat, steamed meatballs, meatballs (beef, chicken, rabbit, turkey), boiled fish, porridge (semolina, rice, buckwheat, oats) in diluted milk, pasta, soft-boiled eggs, steamed omelets, cheese in the form of casserole, kefir, yogurt, soft cheese, jelly, jelly, compotes of sweet berries and , boiled (beets, potatoes, zucchini, pumpkin, cauliflower), raw grated carrots, sweet pears without peel, , baked . It is recommended to eat at least 5 times a day, children 2 years of age 6 -7 times in small portions. 3. Drug therapy: antisecretory and drugs in the presence of the syndrome epigastric pain and prokinetics (domperidone) - (after postprandial distress) syndrome), , enzyme preparations, drugs that correct changes in ANS as needed.

Infant colic - These are sudden and pronounced attacks of crying and anxiety in infants 5 months occurring for no apparent reason, of unknown nature and this is the main cause parental anxiety. Episodes occur more often after eating and in the evening.

Pathogenesis: 1. Dyskinesia of the colon which is more common during or after meals, due to the simultaneous occurrence of two reflexes (gastroileal and gastrocecal) 2. Increased flatulence and distension of the intestinal wall 3. deficiency in infants, as well as gallbladder dysfunction. (Scientists believe that a reduced concentration of cholecystokinin may cause higher excitability of children with colic, as insufficient effect of cholecystokinin).

Diagnostic criteria for infantile colic, Roman criteria IV The diagnosis is established in the presence of all the following symptoms in a child before 5 months of age: 1. Paroxysms of irritability, anxiety or screaming that begin and are terminated without a specific reason and cannot be warned; 2. The duration of episodes is 3 or more hours a day and they appear at least 3 days a week; . 3. There are no signs of disease, fever, and there is no lag in weight gain. Complaints are taken into account mainly from the words of the people caring for the child, and if keep a diary of crying also takes into account the total crying of 3 hours or more during the day Behavior of infants associated with colic: prolonged crying, grimace on the face, which can be regarded as pain, abdominal tension, increased flatulence, flutation and pressing the legs to the abdomen.

Treatment: 1. Creating a calm atmosphere in the family. It is necessary to reassure parents and explain to them that baby colic occurs in most babies, not life-threatening, should soon to pass at the correct care of the child. 2. Rhythmic shaking, stroking 1-3 times a second in silence - can calm down baby, but as soon as you put it - the cry can resume. 3. You can use pressing the baby's tummy to the mother's body, or warm diapers on the abdomen, and wait for the period of crying with the understanding that it will pass. 4. Mother's dietary recommendations: limit fatty foods, foods that cause flatulence (cucumbers, mayonnaise, grapes, , corn), as well as extract (broths, seasonings) substances. also trial, for 14 days, the exclusion of the mother's dairy products, in order assessment of the child's response to cow's milk proteins. 3. At artificial feeding mixes containing as a fatty component short-chain triglycerides are recommended, at the expressed colic use of a mix with partially hydrolyzed protein (more for the purpose of an exception of possible intolerance of proteins of cow's milk) is possible. 4. It is also necessary to make adjustments to the feeding regime of the child: intervals and the number of one feeding may be reduced, but a recommendation is not required. 5. A very important measure is postural therapy and teaching the child on the tummy before feeding, to reduce the amount of gas in the intestine. If the baby's colic occurs against the background of increased flatulence, it is necessary to find the cause of this condition, and to make appropriate correction of dysbiotic or nutritional factors, that determine it. The psychological stress of caring for a baby reduces their ability to calm the child, causes a feeling of alienation and inability to interact with the baby, which can increase the child's arousal. To date, the effectiveness of any drugs and applications has not been proven exhaust tubes for infantile colic.

Functional diarrhea- refers to non-specific chronic diarrhea or diarrhea younger age, which does not violate the general condition of the child and spontaneously disappears at school age.

Diagnostic criteria for functional diarrhea, Roman Consensus IV The diagnosis is established in the presence of all the following symptoms: 1.Painless daily repeated defecation 3 or more times a day with unopened stools. 2. For more than 4 weeks. 3. The onset of symptoms is observed between the ages of 6 and 60 months. 4. Defecation occurs during wakefulness. 5.There is no delay in development if energy needs are adequately met. Does not require treatment.

Functional constipation (FC) - manifested intestinal dysfunction increasing the intervals between bowel movements (compared to the individual norm) or systematic insufficient bowel movement. The symptoms of FC in children are quite diverse and largely depend on the nature pathological conditions that caused them. In some cases, patients are concerned only constipation, in others - they have a large number of different complaints. Frequency defecation can also be different: from once every two to three days to once a week and less often. Some patients have no independent stools. Some children have bowel movements daily, but the act of defecation is difficult, or there are several bowel movements a day in small portions feces, without feeling satisfied after bowel movements. The feces is solid and dry, fragmented, in the form of dry dark balls or lumps, reminiscent of sheep; sometimes it can be - shaped. Prolonged delayed defecation in FC leads to chronic fecal intoxication, which adversely affects the homeostasis of the child's body. In the formation of various complications are important neuro-reflex relationships. There is an increase in autonomic dysfunction, the development of hypochondriac, depressive states, intestinal dysbiosis, hypovitaminosis, decreased immunity, allergic and other diseases. At a long delay of defecation can be observed trauma to the of the during defecation (cracks of the anal canal), as well as the development of reactive inflammation (proctitis, proctosigmoiditis). Therefore, to decide whether a child has functional constipation, it is necessary to use diagnostic criteria.

Diagnostic criteria of functional constipation, Roman criteria IV The diagnosis is established in the presence of children under 4 years of age for at least 1 month 2 of the following signs: 1. Two or fewer bowel movements per week; 2. At least 1 episode per week of incontinence after acquiring hygiene skills; 3.The presence of episodes of excessive restraint of defecation; 4.The presence of painful bowel movements or solid stools; 5. The presence of a large number of fecal masses in the rectum in those accustomed to the toilet children; 6. Large diameter of feces The clinical diagnosis can be made on the basis of the anamnesis and examination of the patient. Instrumental examinations of the rectum are performed after a trial treatment in case of suspicion on anomalies of intestinal development and uncertainty in the diagnosis.

Basic principles of FC treatment: 1. Explaining the problem to the child's parents, providing basic dietary, dietary recommendations that to increase the amount of fluid in the diet, a sufficient amount , which enhances intestinal motility, regular eating and developing a reflex to go to the toilet for a certain period of time (preferably in the morning), and acquaintance with the system of encouragement of a successful act of defecation. 2. Search and elimination of psychological and other causes of constipation 3. The need to achieve soft stools in any way Means of relaxation stools in the manifestation phase can be used for several months, years, they need to be changed, so that there is no addiction. A child with FC needs medical supervision by a general practitioner – family medicine or pediatrician and maintenance therapy for 6-24 months. About recovery indicates the number of bowel movements (more than 6 times a week), otherwise it is necessary to continue therapy Irritable bowel syndrome (IBS) - functional bowel disorder, in which abdominal pain or discomfort is associated with disorders of defecation or intestinal transit (Longstreth et al., 2006).

According to the classification are distinguished • SPK -S (SPK with constipation, constipation) • SPK - D (SPK with diarrhea) • SPK - M (mixed (mixtures) syndrome, diarrhea + constipation) • IBS -A (constipation and diarrhea that intersect (alternate))

Diagnostic criteria of SPK, Roman criteria IV The diagnosis is established in the presence of all the following symptoms: • Abdominal discomfort or pain having 2 or more features for at least 25% of the time: • decreases after defecation; • associated with a change in the frequency of acts of defecation; • associated with a change in the consistency of feces • lack of evidence of inflammatory and metabolic processes, anatomical changes and tumors

According to Roman Criteria III, which have not changed to date, the signs are cumulative confirming the diagnosis of IBS are: 1. Changing the frequency of acts of defecation (4 or more times a day or 2 or less times a week); 2. Change in the consistency of feces (hard, "sheep" or liquid, watery stools); 3. Violation of the act of defecation (stress during defecation, imperative urge to defecate or a feeling of incomplete bowel movement); 4. Excretion of mucus with feces; 5. Flatulence or bloating. 6. The duration of complaints is not less than 2 months

One of the frequent components of the clinical picture of IBS in children is recurrent abdominal pain cavity. It can vary in intensity from mild discomfort, aching pain, that can be tolerated to intense constant cramping pain and even unbearable acute pain that mimics the clinical picture of intestinal obstruction or acute appendicitis. As a rule, the pain is localized in the lower abdomen, more often in the left lower quadrant abdomen in the area of the projection of the descending and sigmoid colon or the left upper quadrant of life. The pain is recurrent and varies in frequency and duration. Periods of exacerbation are most often associated with dietary disorders, stressors, fatigue, etc. For patients with IBS is characterized by the appearance of pain immediately after eating, but most intense pain is observed 1-1.5 hours after eating. Often pain appears on awakening when you need to go to school and is absent during the holidays. On against the background of pain there is bloating, flatulence, increased bowel, diarrhea or other changes in bowel movements. The pain usually subsides after defecation and excretion of gases and do not bother at night. Abdominal pain is associated with severe constant or rhythmic muscle contractions or stretching of the intestine with gas or and both. The second most common complaint in children with IBS is a violation of the frequency and nature of bowel movements. Children may notice a predominance of constipation, diarrhea or their alternation. Adolescents are mostly constipated. At constipations the chair is firm, in the form of small balls. This is characterized by a small daily mass of feces on the type of "sheep feces" (there is no polyfaeces). In addition, with constipation, the patient may complain of a single discharge of feces, which at the beginning of defecation is decorated, and then pasty and even watery, the so-called "cork" stools. Constipation is associated with uncoordinated, non-pulsating contractions of the distal colon, especially the sigmoid colon. If liquid stools predominate, they are usually frequent, in small portions, however, the daily volume remains normal (so-called distal diarrhea of small volume) For patients with IBS is characterized by morning diarrhea that occurs after breakfast and in the first half day, and no diarrhea at night. Both with diarrhea and constipation, mucus may be present, but it is never detected blood if there is no hemorrhoids or sphincteritis. A distinctive feature of the SEC is the variety of complaints, especially vegetative in nature. In addition to intestinal symptoms, children complain of headaches, fatigue, pain in the heart, difficulty breathing, coma when swallowing, nausea, feeling rapid satiety, bloating in the upper abdomen, belching, feeling tremor, urinary incontinence, subfebrile fever. Believe that to the appearance of such complaints leads to a violation of the psychological status of the patient. In part patients with IBS, there are clear symptoms of psychoneurological disorders, most often such as depression, anxiety, phobias, hysteria, panic attacks, hypochondria, somatization syndrome, etc.

Symptoms that exclude the diagnosis of IBS there are so-called "anxiety" symptoms that directly or indirectly indicate the possibility of organic disease (Rasquin et al., 2006): -unmotivated weight loss, persistence of symptoms at night (during sleep), constant intense abdominal pain as the only and main symptom, progression of symptoms, fever, rectal bleeding, painless diarrhea, , lactose intolerance, fructose and gluten, laboratory changes indicators.

Treatment: • Psychocorrection therapy; • Dietary recommendations, depending on the predominance of symptoms. • Restoration of mucosal homeostasis in its violation, by use of • Symptomatic therapy to improve quality of life

Functional abdominal pain. In the clinical picture there are complaints of pain, which more often localized around the navel. Intensity, nature of pain, frequency of attacks diverse. Pain is observed for 6 months or more, with partial or complete lack of connection between pain and food intake, defecation, etc., accompanied slight loss of daily activity, lack of organic causes of pain and diagnostic signs of other functional gastrointestinal disorders. Abdominal migraine - pain of intense, diffuse nature, accompanied nausea, vomiting, diarrhea, paleness and coldness of the extremities, other autonomic manifestations. In children, the pain may be localized in the navel. Duration of pain from 30 minutes up to several hours or even several days. At differential diagnosis with pains of an organic nature it is necessary to take into account the provoking factors characteristic of migraine, young age, family history, increased rate of linear blood flow in the abdomen aorta during Doppler (especially during paroxysm)

6. Materials for self-control. A. Task for self-control 1. Functional gastrointestinal disorders in children include: A. rumination syndrome; B. functional constipation; C. cyclic vomiting syndrome; D. functional dyspepsia; E. all of the above.

2. Diagnostic criteria for cyclic vomiting syndrome include: A. regurgitation (throwing) of gastric contents into the oral cavity, which is either coughed up or chewed and re-slipped; B. bloating due to accumulation of air in the intestines; C. at least 3 typical recurrent severe bouts of vomiting and / or nausea and vomiting; D. rapid saturation; E. discomfort localized in the subcutaneous area along the midline.

3. A 2.5-year-old girl has been suffering from abdominal pain and belching for 1 year, and vomiting has occurred twice in the last month after eating. Which test is most appropriate to verify the diagnosis? A. Fibrogastroduodenoscopy. B. Fractional study of gastric contents. C. Contrast radiography of the . D. Intragastric pH-metry E. Electrogastrography.

4. A 3-year-old boy was diagnosed with functional dyspepsia with increased gastric secretion and increased motility on the basis of clinical and instrumental examination. Which combination of drugs will you choose from the following: A. Ranitidine, gastrocepin, almagel B. Caffeine, famotidine, but-spa C. Euphyllin, famotidine, but-spa D. Proserine, papaverine, almagel E. Valerian, caffeine, but-spa

5. A 3-year-old boy from 6 months of age has constipation. The chair once in 3-4 days, volume and dense. Rectal examination - a large ampoule of the rectum, weak sphincter, feces in the rectal arch. The most likely diagnosis: A. Functional constipation B. Hirschsprung's disease C. Hypercalcemia D. Intestinal obstruction E. Hypothyroidism

6. The child is 1.5 months old, born with a weight of 3250 grams. , 52 cm long, psychophysical development corresponds to age. The actual weight corresponds to age (4350 gr.). The child is breastfed, vomiting is observed periodically. What causes the child's vomiting? A. Aerophagia B. Pylorostenosis C. Pylorospasm D. Acute E. Esophageal atresia

7. To diagnose functional dyspepsia, symptoms should be observed at least: A. 2 weeks; B. 3 weeks; C. 2 months; D. 3 months; E. 6 months.

8. Roman criteria for the third diagnosis of functional dyspepsia in children include: A. there is no evidence of inflammatory, metabolic or neoplastic process, as well as anatomical changes that can explain the signs of the disease; B. symptoms decrease after defecation or are associated with a change in the frequency or nature of stool; C. bloating due to accumulation of air in the intestines; D. two or more periods of intense nausea or persistent vomiting; E. all of the above.

9. The mother of a 3-year-old girl complains of abdominal pain that occurs and worsens after eating, belching sour, frequent constipation, headache, irritability. Ill for 12 months. At reviews girl satisfactory nutrition. Tongue wet with white ossification at the root. The abdomen is soft, painful in the epigastrium. Which of the research methods is most likely to help diagnose the disease? A. Esophagogastroduodenofibroscopy. B. Intragastric pH-metry. C. Fractional study of gastric juice. D. Contrast radioscopy. E. Biochemical analysis of blood.

10. A 2-year-old girl has been ill for 6 months, when vomiting first appeared. Since then, vomiting occurs periodically (3-4 times a day, 2-3 times a month), during vomiting there is abdominal pain and headache. After such attacks, the child is retarded for some time, sleeps a lot. Then the child's condition improves and she feels satisfactory. During the exacerbation of the disease, the mother checked the acetone in the urine, the result was negative. Which diagnosis is most likely? A. Acute gastritis. B. Chronic gastritis. C. Cyclic vomiting syndrome. D. Acetonemic syndrome. E. Neuroarthritic diathesis.

11. The paroxysmal period of cyclic vomiting syndrome continues: A. 24-48 hours (min. 2 hours), but can last for 10 days or more. B. From a few minutes to a few hours. C. From several days to several months. D. All of the above. E. All of the above is incorrect.

12. What remedies are used to buy an attack of vomiting when cyclic vomiting syndrome: A. Cerucal and espumizan. B. Papaverine and cerucal. C. Chlorpromazine and diphenylhydramine. D. Diphenhydramine and analgin. E. Metoclopramide and dufalak. 13. The diagnosis of functional constipation is established in the presence of children under 4 years of age within 1 month of the following symptoms: A. The presence of episodes of delayed defecation. B. The presence of painful bowel movements or solid stools. C. Two or fewer bowel movements per week. D. At least 1 episode a week of incontinence after acquiring hygiene skills. E. All of the above.

14. Children with functional constipation are prescribed the following diet: A. Diet №1. B. Diet №2. C. Diet №3. D. Diet №4. E. Diet №5.

B. Tasks for self-control: 1. The mother of a 3-year-old girl complains of poor appetite in the child, abdominal pain, nausea. Pain occurs without connection with food intake, often in the morning, aching, prolonged, without clear localization, passes in state of rest. Complaints last about 4 months. 1. Make a preliminary diagnosis. 2. Make a survey plan. 3. Make a treatment plan. 4. Write this child a motilium.

2. The child is 2.5 years old. Ill for a year. Complaints of poor appetite, intermittent vomiting that is not related to food. Vomiting occurs 2-3 times over time, with bile impurities. After nausea, the child is retarded, sleepy. The duration of the paroxysmal period of vomiting is from 3 to 10 days. After that, the condition improves, the child becomes active, appetite is restored. On examination, the child's condition is moderate. The skin and visible mucous membranes are pale. Nasal breathing is free. The pharynx is not hyperemic. Above the lungs vesicular respiration. Heart tones are pure, rhythmic. The abdomen is soft, painless. Physiological stools are normal. 1. Make a preliminary diagnosis. 2. Make a survey plan. 3. Make a treatment plan. 4. Write this child phospholugel

Literature: Basic literature: 1. Behrman Richard E., Robert M. Kliegman, Hal B. Jenson, eds. Nelson Texbook of Paediatrics. Philadelfia: WB Saunders, 18th edition - 2012. – 994 р. – Р.638. 2. Buryak V.N., Makhmutov R.F., Poshehonova J.V. Children`s diseases. – Donetsk: Nord-Press, 2010. – 328 p. 3. Pediatry. Guidance aid / Ed. by O. Tiazhka – Kyiv: Medicine, 2007. 4. Pediatrics. Ed. Kryuchko TO, Abaturova OE National textbook for students of dental faculties of higher educational institutions of the Ministry of Health of Ukraine, Kyiv: "Health", 2015. - 206 p. Additional literature: 1. Illustrated Textbook of Paediatrics/ Ed.: T. Lissauer, G. Clayden.- Edinburg: Mosby, 2006.- 410 pp. 2. Forfar & Arneil’s Textbook of Pediatrics/ Ed.: N.Mc Intosh, P.J. Helms, R.L. Smyth, S. Logan.- Edinburg: Churchill Livingstone, 2008. - 1687 pp. 3. Further Reading Health Care Guideline: Diagnosis and Treatment of Respiratory Illness in Children and Adults INSTITUTE FOR CLINICAL SYSTEMS IMPROVEMENT Second Edition January 2008. Information resources http://www.booksmed.com/pediatriya http://pediatriya.info http://health-ua.com/parts/pediatrics http://www.med-edu.ru/pediatr http://medi.ru/Doc/j01.htm http://www.mif-ua.com/archive/zhurnal-zdorove-rebenka/numbers http://medkniga.ucoz.net/publ/pediatrija/40 http://www.medport.info/index.php?option=com_content&view=section&id=48&Itemid=73

The methodical instructions are compiled by Associate Prof Poda O.A.

Methodical instructions were discussed and approved at the meeting of department of Pediatrics №2 protocol № ___ of ______20____.

Additions and changes were made “____” ______20____ Additions and changes were made “____” ______20____ Additions and changes were made “____” ______20____ Additions and changes were made “____” ______20____ Additions and changes were made “____” ______20____ MINISTRY OF HEALTH OF UKRAINE UKRAINIAN MEDICAL STOMATOLOGICAL ACADEMY

Approved at the meeting of department of Pediatrics №2 Minutes № 21 " 05 " 06 2020 Head of the department MD, Prof. Kryuchko T.O. ______

METHODICAL RECOMMENDATIONS INDIVIDUAL WORK OF STUDENTS IN PREPARATION FOR THE PRACTICAL (SEMINARS) CLASS

Academic discipline Pediatrics Моdule № 1 The most common somatic diseases in children Topic of lesson Rickets. Protein-energy malnutrition in children. Course 4 Faculty Medical

Poltava-2020 1. Relevance of the topic: Rickets - a disease of early childhood. Usually this disease begins during the most intensive growth of the child - up to 1 year (which is why rickets is also called growth disease), and often leaves consequences for life. Rickets is most often diagnosed at the II-III degree of severity. Mild forms of the disease go unnoticed, so if you do not look too closely at the child - they can be easily missed. According to rough data, rickets affects about 50% of children, 7-10% of them suffer from severe and moderate rickets. Rickets refers to social diseases, because its frequency and severity are directly related to socio-economic and hygienic living conditions, cultural standards of the population, the peculiarities of breastfeeding. Transferred in early childhood rickets adversely affects the development of the child in the following age periods: long-term residual deformities of the skeletal system, muscle weakness and ligaments, there are improper eruption of teeth. Therefore, the doctor, regardless of specialty, must be able to diagnose rickets in time, adequately treat and take preventive measures.

2. Specific objectives: -Analyze: etiopathogenesis of rickets in children and principles of treatment of rickets in children; -Explain: the rules for prescribing drugs for rickets in children -Offer: to collect anamnesis from the parents and conduct a general examination and systematic examination of the child, pay attention to changes in the system; -Classify: rickets in children; -Interpret: the diagnosis, formulate it in accordance with modern classification; -Analyze: the results of laboratory and instrumental examinations; -Draw up: make a plan for an auxiliary examination of the child, evaluate its results and make a plan for the treatment of the child.

3. Basic knowledge, skills, abilities necessary for studying the topic (interdisciplinary integration): № Names of previous disciplines Acquired skills 1 Normal anatomy Know the anatomical features of the gastrointestinal tract in children 2 Normal physiology Have an idea of the physiological processes that occur in the digestive tract in children. 3 Pathological physiology Identify pathophysiological processes that occur in the gastrointestinal tract on the basis of symptoms 4 Microbiology with virology and Have knowledge of the normal composition of the immunology intestinal microflora of newborns and young children. 5 Patient care and nursing practice Demonstrate skills in caring for young children with functional disorders of the gastrointestinal tract and providing pre-medical care. 6 Propaedeutic pediatrics To have knowledge about the main clinical symptoms of functional gastrointestinal disorders in young children, to have an idea of laboratory and instrumental methods of examination. 7 Social medicine and health care Apply knowledge about the structure of health care for organization children for the proper use of resources of the health care system in terms of treatment and prevention of functional disorders of the gastrointestinal tract.

4. Tasks for independent work in preparation for the lesson. 4.1. The list of the basic terms, parameters, characteristics which the student should master at preparation for employment № Term Definition 1 Rickets disease of early age children which occurs because of the deficiency or disturbance of exchange of D; it is accompanied by the disorders of phosphor-calcium exchange and manifested clinically through the lesion of tissue in the form of osteomalacia, hyperplasia of osteoid tissue, disturbance of endochondral ossification, lesion of muscular system and internal organs. 2 Hypervitaminosis D Excessive administration of vitamin D with resulting hypercalcemia is a serious condition which should be prevented. 3 Rachitic tetany (spasmophilia) disease, which is stipulated by the disturbance of with the decrease of calcium content in blood (>2 mmol/l), characterized by the susceptibility to high neuromuscular excitability and development of convulsive attacks. 4 Hypotrophy pathological state, which is characterized by the retardation of body weight and height, occurs as the result of chronic disturbance of nutrition caused by insufficient supply of nutrients and their digestion.

4.2. Theoretical questions for the lesson: 1. Definition of "rickets", "spasmophilia", "convulsions". 2. The main causes of rickets and spasmophilia in young children. 3. Classification of rickets and spasmophilia in children. 4. Clinical and laboratory characteristics of rickets depending on the course and severity of the disease. 5. Differential diagnosis of rickets. 6. Pathogenesis and clinical manifestations of spasmophilia. 7. Principles of rational therapy and prevention of rickets in children. 8. Emergency care for convulsive syndrome in children. 9. Characterize the degrees of hypotrophy in children. 10. Determine the treatment tactics and prophylaxis of dystrophy in children. 4.3. Practical work (tasks) performed in class: 1. Work with test tasks. 2. The work of students in the wards at the bedside of children with rickets and hypotrophy A) Collection of complaints, medical history; B) Objective examination of children; C) Formulation of the diagnosis according to the modern classification; D) Make a plan for the examination of a sick child; E) Make a treatment plan for a sick child. 3. Clinical analysis of an indicative case. 4. Solving situational cases. .

5. Contents of the topic:

Rickets – disease of early age children which occurs because of the deficiency or disturbance of exchange of vitamin D; it is accompanied by the disorders of phosphor-calcium exchange and manifested clinically through the lesion of bone tissue in the form of osteomalacia, hyperplasia of osteoid tissue, disturbance of endochondral ossification, lesion of muscular system and internal organs. Epidemiology. The disease is more often registered in north nations which live under the conditions of sunlight deficiency. Also, rickets is the serious problem for dark-skinned children, even in the sunniest countries of the world; it is connected with the cultural peculiarities of restriction of sun impact and absence of additives of vitamin D in breastfeeding women. It should be noted that for the transformation of biologically active vitamin D people with dark skin need more exposure to the sun irradiation than people with light skin. In the beginning of the 20th century, rickets was diagnosed in 50-80% of early-age children in the countries of Western Europe and up to 70% of children – in Ukraine. In developed countries, where the specific vitamin prophylaxis and vitaminization of infant foodstuffs are organized, the severe forms of rickets became rarity but subclinical and X-ray manifestations of this disease remain widespread. This disease has the most severe form in premature children. Etiology. Among the causing factors, the following factors are distinguished: exogenous factors – insufficient supply of vitamin D with food, unbalanced nutrition; insufficient formation of vitamin D in skin under the impact of ultraviolet; and endogenous factors – disturbance of vitamin D absorption due to the diseases of intestine, kidneys etc. (gluten enteropathy, cystic fibrosis, tubulopathy). Classification. Classification of rickets in children (according to O.M. Lukyanova, U.G. Antypkin, L.I. Omelchenko, 1991). Disease Severity degree Clinical variants Disease periods progression • Acute • Mild • Hypocalciemic • Primary • Subacute • Moderately • Hypophosphatemic • Eruptive phase • Recurrent severe • Without significant • Recovery • Severe deviations of calcium and • Residual effects phosphor content in blood Severity degrees: - mild (1st degree) – diagnosed in case of lesion of 1-2 skeleton sections; - moderately severe (2nd degree) – lesion of more than 2 skeleton sections and presence of muscular hypotonia; - severe (3rd degree) – lesion of all skeleton sections, presence of muscular hypotonia and lesion of internal organs. Clinics. Primary period (during the whole first year of life, more often in 2-3 months) is manifested through excessive sweating, occiput baldness, anxiety, muscular hypotonia; insignificant softening of edges of big fontanel. Eruptive phase (4th-6th month of life) is characterized by the symptoms of nervous system suppression; alterations of skull , chest and extremities bones occur. Disturbance of the functions of internal organs, static functions, muscular hypotonia are detected. Laboratory signs: anemia, hypocalcemia (norm 2.25-2.5 mmol/l), hypophosphatemia (norm 1.45-2.1 mmol/l), increase of activity of alkaline phosphatase in blood serum. Period of residual effects (2nd-3rd year of life): there are consequences only in the form of bone deformations, sometimes anemia.

Severity degrees of rickets. Differential diagnostics. In case of retention of clinical symptomatic and absence of efficient treatment, rickets-like diseases, chondrodystrophy, congenital fragility of bones, hypothyroidism, hypophosphatasia are excluded. Treatment. Medicine with vitamin D3, calcium, , carnitine chloride, orotic acid, ATP, polyvitamins (with the adequate content of of groups B, C, E according to age needs) are applied. In 2-3 weeks from the beginning of drug therapy, the full-body massage, therapeutic physical training, baths: pine baths (mainly for the children with increased neuro-reflectory excitability), salt baths (for sedentary children) are prescribed.

Prescription of vitamin D3 for therapeutic purposes. Severity Daily dose, Duration of intake degrees IU Mild 2000 During 1-1.5 months, then for the prevention of exacerbation and recurrence of the disease 2000 IU during 30 days, 2-3 times a year, with the intervals between them Moderately 4000 of not less than 3 months till the child reaches the age of 3-5 years severe 5000 Severe

Prevention. General principles include the observation of day regime and taking sanitary and hygienic measures. Maintenance of breastfeeding is also important, in case if it is impossible – use of highly-adapted milk formulas.

Specific prevention of rickets. Groups of Period of Daily dose of Duration of intake of vitamin D3 women and commencement of vitamin D3 children specific prevention Antenatal prophylaxis Healthy From 28-32 weeks of 500 IU Every day, during 6-8 weeks pregnant women pregnancy Postnatal prophylaxis Variant 1. 500 IU Every day, during 3 years except for 3 nd Mature healthy On the 2 month of life summer months (course dose per year – children 180 000 IU) Variant 2. 2000 IU Every day, during 30 days. Then, up to the On the 2nd, 6th, 10th age of 3 years 2-3 courses per year with months of life the intervals between them of 3 months (course dose per year - 180 000 IU)

Prognosis. In case of timely treatment – favorable prognosis; serious prognosis – in case of combination with tetany. Laryngospasm and convulsion can result in negative consequences. Skeleton deformations which are observed in the majority of children, who had rickets, disappear through the years.

Rachitic tetany (spasmophilia) – disease, which is stipulated by the disturbance of mineral metabolism with the decrease of calcium content in blood (>2 mmol/l), characterized by the susceptibility to high neuromuscular excitability and development of convulsive attacks. Epidemiology. Pathology is detected in 3.5-4% of children at the age of 6 months to 18 months. Etiology. Disease is closely connected with rickets. Etiological factors include the deficiency of calcium in the diet, hypervitaminosis D. Anamnestically, hypoxic-traumatic lesions of the central nervous system take place. Pathogenesis. Increase of neuromuscular excitability caused by hypocalcemia results in the convulsive syndrome. The provoking factors can include prolonged cry, invasive manipulations, rise of body temperature, fright etc. Biochemical changes relate to phosphor-calcium metabolism – deficiency of vitamin D results in hypocalcemia, relative hyperphosphatemia. Frequent vomiting, can cause the quick shift of acid-alkaline balance towards alkalosis and provoke the convulsive attacks. Classification. There are manifest and latent forms of rachitic tetany. Clinics.

Clinics of rachitic tetany. Plasma biochemical parameters in children with rachitic tetany. Latent form Manfest form Norm Calcium - ionized >0.75-1 mmol/l >0.75 mmol/l 1.29-1.31 mmol/l - total >1.75-1.88 mmol/l >1.75 mmol/l 2.25 – 2.5 mmol/l рН blood 7.65 (alkalosis) 7.35

Changes on ECG are represented by the extension of QT interval, arrhythmia. Laryngospasm (duration - several sec. to 1-2 min., possible repetition up to 20 times/day): - Child has frightened facial expression; eyes are widely open; there is strangled (“cock”) cry when exhaling; - Loud groaning cry → constrained inhalation → apnea → several loud sibilant inhalations at the end of the fit; - Sudden paleness of skin, of lips; - Motor anxiety with tossing back of the head, tension of body and extremities, bulging of big sinciput in infants. Carpopedal spasm (duration – several minutes to several hours, days): - Usually observed in children at the age of 1 year and older; - Manifested in the form of tonic contractions of hands and feet; - Hands take the position of “obstetrician’s hand”, feet – in the position of sudden plantar flexion; - If spasm lingers, edema is developed on the back side of hands and feet; - Tonic convulsions of different groups of muscles (respiratory muscles, muscles of urinary bladder, intestine) can take place as well. Convulsions (duration – 2-3 min.) – the hardest manifestation of rachitic tetany. Clonic and tonic convulsions of facial muscles at first, then – body muscles, muscles of extremities occur. Convulsions are the consequence of cerebral dysrhythmia as a result of increased neuromuscular excitability. During the fit, child loses consciousness; after the fit child falls asleep. Treatment and emergency care.

HYPERVITAMINOSIS D - Excessive administration of vitamin D with resulting hypercalcemia is a serious condition which should be prevented. Clinical manifestations - The patient feels weak, thirsty, anorexic and loses weight - Nausea, vomiting and polyuria - Polydipsia and polyuria. - Constipation. - . - Kidneys: stone formation or nephrocalcinosis (deposition of calcium in kidney parenchyma) which may lead to renal failure. - Deposition of calcium in soft tissues around the and in the walls of blood vessels. Metastatic calcification may occur in the heart, lungs, thyroid and pancreas. Laboratory findings: Serum calcium increases. Serum phosphorus is normal. It increases in renal failure. Urinary calcium increases. Radiological findings: - Increased density of bones in the growing areas. - Dense metaphyseal lines. - Calcium deposition in soft tissues. - Ultrasonography for renal stones or nephrocalcinosis. Treatment: − Stop administration of vitamin D. − Correct dehydration. − Decrease calcium in diet by stopping the usage of milk and its products or by increasing cereals in the diet to decrease calcium absorption. − Prednisone 2 mg/kg/day is an effective , it should be given until the serum calcium level falls to 12 mg/dl, and then it should be stopped. Hypotrophy (protein-energy insufficiency) – pathological state, which is characterized by the retardation of body weight and height, occurs as the result of chronic disturbance of nutrition caused by insufficient supply of nutrients and their digestion. Epidemiology. According to WHO data, in developing countries up to 20-30% of early age children have chronic disorders of nutrition, in particular – protein-energy insufficiency. Etiology. Exogenous factors: • Insufficient supply of nutrients, hypovitaminosis; • Hypogalactia of mother; • Feeding with low-quality milk formulas, late introduction of supplementary food; • Defects of care, social deprivation; • Infectious diseases (intestine , pyelonephritis, pheumonia, intrauterine infections, sepsis); • Intoxications (nitrate, hypervitaminosis D and A). Endogenous factors: • Malabsorption syndrome (enzymopathy, cystic fibrosis, gluten enteropathy); • Pathology of (bronchopulmonary dysplasia); • Encephalopathy (hypoxic lesion of the central nervous system, hemorrhage, infantile cerebral paralysis, hydrocephaly, microcephaly); • Endocrine diseases (hypothyroidism, adrenogenital syndrome, pituitary dwarfism); • Hereditary diseases (Down syndrome); • Immune deficiency state (hereditary, secondary); • Congenital disorders (pyloric stenosis, cleft upper lip and hard palate, congenital heart defect, cardiopathy); • Malignant neoplasms. Pathogenesis depends on the causes which resulted in the disease. Gradually, the metabolism disturbance is accumulated; protein catabolism increases and protein synthesis is reduced. Exhaustion of stores of fat, carbohydrates, essential microelements results in the disturbance of functioning with the reduction of organism resistibility to respiratory infections; disturbance of neuropsychic development is accompanied by the delay of language development, cognitive functions and skills. Classification.

Differential diagnostics is carried out with the defects of development of gastrointestinal tract, congenital heart disease, dwarfism, chondrodystrophy, kidney diseases. Treatment. In case of hypotrophy of the 1st degree, the treatment is carried out on outpatient basis: efficient nutrition according to the age, vitamins, enzymatic drugs, probiotics and prebiotics (during 1 month), massage. Treatment of hypotrophy of the 2nd and 3rd degrees is carried out in hospital: - Parenteral feeding is prescribed in case of severe degree and malabsorption syndrome (solution of amino acids, fat emulsions); - Diet therapy with the prescription of specialized medical milk and milkless mixtures, medical products based on soy protein isolate, protein hydrolysate, in small portions, up to 8-12 times per day; - Consumption of amino acids (methionine, arginine aspartate), vitamins and vitamin-like drugs, macro- and microelements; - Correction of dysbiosis (probiotics – bifidumbacterinum, lactobacterinum etc.); - Physiotherapy (electrophoresis, paraffin bath on abdomen area); - General massage, up to 20 procedures per course. Prognosis. Hypotrophy development during the first 3 months of life differs by worse prognosis in comparison with the children of older age. In case of combination with intercurrent, infectious diseases, various complications, the prognosis becomes worse in hypotrophy of any degree.

6. Materials for self-control. A. Task for self-control 1. The newborn mature child, who was born without complications, has cleft upper lip. Child’s mother has pronounced herpetic rash on the lips. What primary tactics of child feeding will be correct? A. Breastfeeding can be started B. Feeding with expressed breast milk from spoon C. Child feeding through the tube with milk formulas D. Feeding with milk formulas from spoon E. Child feeding through the tube with expressed breast milk 2. Twins at the age of 5 months, who were born premature and had improper feeding, are diagnosed rd with the symptoms of rickets of the 3 degree. What daily dose of vitamin D3 should be prescribed for the purposes of treatment: A. 4 000 IU B. 10 000 IU C. 5 000 IU D. 2 000 IU E. 8 000 IU 3. Mother of 4-months old child, who is fed artificially, visited the doctor in order to receive advice on the age of introduction of supplementary food. Child was born with the weight of 3200 g, current weight of the child is 6650 g. What age is correct, in your opinion? A. At the age of 7 months B. At the age of 5 months C. At the age of 5.5 months D. At the age of 6 months E. At the age of 4.5 months 4. 10-months old girl has apnea fit along with the symptoms of rickets against the background of cry with the following loud sibilant inhalations (“cock cry”). The child is anxious, frightened, body and extremities are tense, head is slightly tossed back. Skin is pale, lips are cyanotic. In blood analysis: calcium – 0.87 mmol/l, phosphor – 1.4 mmol/l, alkaline phosphatase is high. What state does this clinical pattern correspond to? A. Latent form of rachitic tetany B. Carpopedal spasm C. Convulsions D. Hidden form of rachitic tetany E. Laryngospasm 5. Mother of 4-months old child, who is breastfed, complains of the child’s anxiety between the feedings, during sleep. Mother notes the reduction of lactation for the last month. For the last month the child has gained 300 g. The most informative procedure concerning the determination of deficiency of breast milk is: A. Check weighing B. Expression of milk in the beginning of the next feeding C. Determination of the number of urinations for the day D. Determination of the frequency of of child E. Number of child feedings per day 6. 10-months boy who was born at the gestation age of 37 weeks, with the weight of 2500 g is at the doctor’s office. Examination: body weight 6300 g, child does not sit, there are no teeth. Skin is dry, pale; subcutaneous tissue is narrowed on the body, extremities and face. There are candidiasis effects in oral cavity. Clinical pattern corresponds to hypotrophy: A. Congenital hypotrophy of the 3rd degree B. Acquired hypotrophy of the 1st degree C. Acquired hypotrophy of the 3rd degree D. Congenital hypotrophy of the 2nd degree E. Acquired hypotrophy of the 2nd degree 7. Parents of 4-months old boy complain of anxious sleep of child, shuddering caused by insignificant sounds, pronounced excessive sweatiness at night. For the last 3 weeks the reduction of appetite has been noted. Examination: baldness of occiput, insignificant flattening of occiput, muscular dystonia. What diagnosis does this clinical pattern correspond to? A. Moderately severe rickets, eruptive phase B. Moderately severe rickets, period of residual effects C. Mild rickets, eruptive period D. Mild rickets, primary period E. Mild rickets, period of residual effects 8. Mother of 3-months old infant complains of child anxiety occurring in 1 hour after feeding. Infant cries, sucks fingers, refuses to have water which mother offers. Child weight at birth is 3700 g, at the moment of examination – 5750 g; for the last month the child has gained 650 g in weight. Child has been fed with milk formula from birth. Determine the daily volume of milk formula for feeding. A. 960 ml B. 820 ml C. 640 ml D. 720 ml E. 1150 ml 9. 9-months old child with present symptoms of rickets during cry suddenly starts having loud respiration, voice hoarseness, cyanosis, short-term cessation of breathing, then – convulsions in extremities with tonic character during 15-20 s. In 10 min. the child’s state is satisfactory, activity and adequacy are restored. What listed medicine must be prescribed to the child primarily? A. B. Diazepamum C. Sodium oxybutyrate D. Calcium gluconate E. Vitamin D3 10. Feeding mother has the symptoms of atopic dermatitis. 3-months old girl has been breastfed from birth. Parameters of physical development are in norm. What tactics in relation to child should be selected? A. Prescribe antiallergic medicine to mother B. Use mixed feeding for child C. Prescribe antiallergic medicine to child D. Continue breastfeeding E. Continue feeding with highly adapted milk formulas

B. Case for self-control: Case 1. Parents with 6-months old child came to the doctor for scheduled examination. Girl was born mature, with body weight of 3750 g; child is breastfed. Rickets prevention was being performed on the nd th 2 to the 4 month, then vitamin D3 was not administered due to the adequate nutrition of feeding mother. At the examination, the child’s body weight is 7030 g, for the last month child has gained 300 g. Mother notes excessive sweatiness in child during the night time, anxiety during the period between feedings (child sleeps for 1-1.5 hours, after waking up child cries). During feeding, after 5-7 minutes of sucking the child cries again, attempts to continue feeding do not help to calm down the child. Objectively: skin and mucous membranes are clear, turgor of tissues is slightly reduced; decrease of adipose layer on abdomen is observed. On occiput – baldness, big fontanel has insignificant softening on the edges. Task: 1. Formulate the diagnosis according to classification. 2. Plan further feeding of child. 3. What treatment should be reasonably prescribed to child? 4. Plan prophylactic measures.

Case 2. 13-months old girl, who was born premature and was not fed adequately, is examined for rickets. Mother complains of periodic fits for the last week which are manifested by convulsive contractions of hands and feet and take position of sudden flexion. Child’s consciousness is not disturbed; urinary retention (up to 4 hours) and constipations take place periodically. Examination, in blood analysis: erythrocytes 3.0х1012/l, Hb – 105 g/l, leucocytes – 6.4х109/l, calcium – 0.82 mmol/l, phosphor – 1.1 mmol/l, increase of alkaline phosphatase. Task: 1. What state does this clinical pattern of disease correspond to? 2. Evaluate the results of hematologic study. 3. Plan the remedial actions for child.

Literature: Basic literature: 1. Behrman Richard E., Robert M. Kliegman, Hal B. Jenson, eds. Nelson Texbook of Paediatrics. Philadelfia: WB Saunders, 18th edition - 2012. – 994 р. – Р.638. 2. Buryak V.N., Makhmutov R.F., Poshehonova J.V. Children`s diseases. – Donetsk: Nord-Press, 2010. – 328 p. 3. Pediatry. Guidance aid / Ed. by O. Tiazhka – Kyiv: Medicine, 2007. 4. Pediatrics. Ed. Kryuchko TO, Abaturova OE National textbook for students of dental faculties of higher educational institutions of the Ministry of Health of Ukraine, Kyiv: "Health", 2015. - 206 p. Additional literature: 1. Illustrated Textbook of Paediatrics/ Ed.: T. Lissauer, G. Clayden.- Edinburg: Mosby, 2006.- 410 pp. 2. Forfar & Arneil’s Textbook of Pediatrics/ Ed.: N.Mc Intosh, P.J. Helms, R.L. Smyth, S. Logan.- Edinburg: Churchill Livingstone, 2008. - 1687 pp. 3. Further Reading Health Care Guideline: Diagnosis and Treatment of Respiratory Illness in Children and Adults INSTITUTE FOR CLINICAL SYSTEMS IMPROVEMENT Second Edition January 2008. Information resources http://www.booksmed.com/pediatriya http://pediatriya.info http://health-ua.com/parts/pediatrics http://www.med-edu.ru/pediatr http://medi.ru/Doc/j01.htm http://www.mif-ua.com/archive/zhurnal-zdorove-rebenka/numbers http://medkniga.ucoz.net/publ/pediatrija/40 http://www.medport.info/index.php?option=com_content&view=section&id=48&Itemid=73

The methodical instructions are compiled by Associate Prof Poda O.A.\

Methodical instructions were discussed and approved at the meeting of department of Pediatrics №2 protocol № ___ of ______20____. Additions and changes were made “____” ______20____

Additions and changes were made “____” ______20____ Additions and changes were made “____” ______20____ Additions and changes were made “____” ______20____ Additions and changes were made “____” ______20____ Ministry of Health of Ukraine Ukrainian Medical Stomatological Academy

Approved at the meeting of department of Pediatrics №2 Minutes № 21 " 05 " 06 2020 Head of the department MD, Prof. Kryuchko T.O. ______

METHODICAL RECOMMENDATIONS FOR INDIVIDUAL WORK OF STUDENTS IN PREPARATION FOR THE PRACTICAL ( SEMINARS) CLASS

Academic discipline Pediatrics Module № 1 Substantial module № 2

Topic of lesson Acute respiratory infections in children: acute nasopharyngitis, acute pharyngitis, acute laryngopharyngitis, acute tracheitis. Acute in children. Course IV

Faculty Medical

Poltava 2020

1. Actuality of the subject.

Acute respiratory infections (ARIs) are the leading cause of death in children under the age of five worldwide, and most of these deaths are caused by bronchiolitis and pneumonia. In children, the most common cause of bronchitis is a virus, although in children over 6 years of age, it can be caused by bacteria. Acute bronchitis is usually a mild condition. Acute bronchitis may follow the common cold or other viral infections in the upper respiratory tract. It may also occur in children with chronic sinusitis, allergies, or those with enlarged tonsils and adenoids. Pneumonia is a complication that can follow bronchitis.

2. Concrete aims:  Define the patient's complaints.  Collect anamnesis (history) disease.  Be able to conduct the chest palpation, and of lungs.  Know classification of acute respiratory infections  Make up a plan of investigation of a child with acute respiratory infections  Be able to estimate general and biochemical blood analysis, nose and throat secretion investigation, pleural fluid investigation, to interpret functional lung investigations (spirography,oxygenometria), X-ray investigation.  Make a preliminary diagnosis of acute respiratory infections  Give a basic, pathogenetic and symptomatic therapy acute respiratory infections  To demonstrate the usage by medical specialist of moral-deontological principles in child's pulmonology. 3. Basic knowledge, skills, habits necessary for studying the subject (interdisciplinary integration). Names of previous disciplines Acquired skills and habits

1. Anatomy, histology Аnatomic and histological features of respiratory system at children

2. Physiology Peculiarities of the respiratory system and gaseous exchange in child organism in norm and pathology.

3. Biochemistry To give a clinical estimation of the changes of biochemical indexes of blood.

4. Propadeutic pediatrics Characteristic complaints, clinical syndromes at the diseases of the respiratory system. Ability to collect anamnesis, find out the special complaints, conduct the clinical examination of children with the diseases of the respiratory system.

5. Pharmacology To have knowledge about the basic groups of medical preparations which are used at treatment of pneumonia.

6. Public Health To use knowledge about the structure of medical help for child's population for the proper use of resources of the system of health protection in the plan of prophylaxis and treatment.

4. Tasks for independent work during preparation for the study:

4.1. The enumeration of the main terms, parameters, characteristics which the student is to assimilate while preparing for the class:

Term Definition

Names of previous Acquired skills and habits Disciplines

Common Cold Acute viral inflammation of the mucous membrane of the nose and may be the pharynx. Influenza-Like Fever, pharyngitis, adenoiditis, enlarged tender cervical lymph nodes and Disease hoarse voice with scattered rhonchi in the chest. Acute The common causative organisms are group A B hemolytic streptococci Tonsillopharyngitis and the adenoviruses Harsh low pitched respiratory sound produced by upper respiratory obstruction mainly in the laryngeal area.

Cyanosis Rate of oxygen deficiency in the blood

Respiratory rate Newborn - 40 - 60, 1 year 30 - 35, 5 years - 25, 10 years - 20, over 12 (bpm) years - 20 - 16 Dyspnea with a violation of its frequency, depth and rhythm

Types of inspirator, expiratory, mixed breathlessness

Types rales Moist, wheezing 4.2. Theoretical questions for the class:

1. What is acute virus infections? 2. What factors influence on the development and expression of acute virus infections? 3. What are the mechanisms of acute virus infections? 4. What are the symptoms of acute virus infections? 5. What will you see during the physical examination of acute virus infections? 6. What is the classification of acute virus infections? 7. Let’s speak about the acute virus infections treatment. What the routes of administration are the most popular in children? 8. What is bronchitis? What is bronchiolitis? 9. What is acute bronchitis? 10. What causes acute bronchitis, bronchiolitis? 11. Pathogenesis of bronchitis. 12. Classification of bronchitis in children. 13. What are the symptoms of acute bronchitis? 14. How is acute bronchitis diagnosed? 15. Laboratory diagnostics of bronchitis in children. 16. Evaluation of the functional state of lungs. 17. Principles of treatment of bronchitis in children ( Therapy, expectorants, antipyretics). 18. Peculiarities of infusion therapy in bronchiolitis in children.

4.3. Practical tasks to be carried out in class: 1. Working with test tasks. 2. Work of students in chambers near the bed of sick children with acute virus infections, acute bronchitis. 3. Interpretation of additional methods of examination. 4. Clinical analysis of model case. 5. Deciding of situation tasks. 6. Setting of treatment for every child with acute virus infections.

5. Content of the topic:

COMMON COLD (ACUTE RHINITIS) Acute viral inflammation of the mucous membrane of the nose and may be the pharynx. Etiology : Rhinoviruses are the common cause (they are about 90 serotype). Clinical Picture : – Low grade fever may be present. – Watery nasal discharge. – Sneezing. – . – Nasal tone of voice. – . – Nasal obstruction may interfere with suckling and cause irritability of the infant. Treatment: – No specific treatment. – The condition is self limited. – Nasal drops may be used in complete nasal obstruction (but should be used for a short period e.g. ephedrine nasal drops). – Nursing mothers with cold should put masks on their mouth and nose. INFLUENZA-LIKE DISEASE Fever, pharyngitis, adenoiditis, enlarged tender cervical lymph nodes and hoarse voice with scattered rhonchi in the chest. Etiology : Mainly viral as : *Adenovirus. * Rhinoviruses. *Parainfluenza. * Respiratory syncytial viruses. Treatment: – No specific treatment. – Only antipyretics. N.B. The commonest cause of cough in children is upper respiratory tract infection. ACUTE TONSILLOPHARYNGITIS The common causative organisms are group A B hemolytic streptococci and the adenoviruses. Clinical picture : Fever (which may cause febrile convulsions) usually of acute onset associated with anorexia ± vomiting + dysphagia + thick voice. Examination : – The tonsils are enlarged, red showing follicles of pus or pseudorrrernbrane – Inspection of the oropharynx may reveal purulent mucus on the posterior wall representing drainage from the adenoids, nasopharynx and/or paranasal sinuses. The lymph follicles on the posterior wall are hypertrophied and appear as red inflammed elevations of few to several millimeters diameters. – The cervical lymph nodes are enlarged and tender. Bacterial Viral Onset Sudden Gradual Course Severe Mild Temperature Cough High Late Both Moderate Early Follicles and/or membrane Cervical + ± membrane nodes >10,000 ± WBCs ++ < 10,000 Complications as quinzy ±

Treatment of acute tonsillopharyngitis: Bacterial: General: – Antipyretics – Enough fluid intake.

Specifi–c: Penicillin is the drug of choice 25,000-50,000 U/kg/day (procaine penicillin I.M. re injection/day for 10 days). – If sensitive to penicillin, erythromycin is given for 10 days.

Viral: – Only symptomatic treatment. – No specific treatment Indications of tonsillectomy : 1. In children who have 4 or more culture proved episodes of group A strept. pharyngitis associated with tonsillitis in a year. "Frequent sore throat is not a valid indication". N.R Normally, tonsils are relatively larger during childhood than in later years. 2. Chronic hypertrophy of the tonsils obstructing swallowing or breathing. 3. Peritonsillar abscess (quinzy). N.B. No systemic disturbance is an indication for tonsillectomy. This applies to children with rheumatic fever and glomerulonephritis. Contraindications for tonsillectomy and/or adenoidectomy: 1. Absolute Hemophilia and other disorders of and agranulocytosis. 2. Relative: – Diabetes. – Congestive heart failure. – Advanced T.B. – Active rheumatic fever. – Active glomerulonephritis. – Nephrotic syndrome. Indications for adenoidectomy 1. Chronic infection causing repeated or long standing otitis media, sinusitis or pyrexia because of the retained pus in their crypts. 2. Huge obstructive adenoids which cause mouth breathing and/or early impairment of hearing. Age for operation (tonsillectomy and adenoidectomy) : − 4-5 years. Important notes on the operation : 1. Never operate during an epidemic of poliomyelitis. 2. The operation should be postponed until 2-3 weeks after subsidence of an infection. STRIDOR Definition: Harsh low pitched respiratory sound produced by upper respiratory obstruction mainly in the laryngeal area. Causes : I Acute : A. Infections: – Viral croup: acute laryngotracheobronchitis. – Acute epiglottitis. – Diphtheritic laryngitis. – Bacterial tracheitis. B. Allergy: – Angioneurotic edema. – Acute spasmodic laryngitis (mid-night croup). C. Mechanical. – Laryngeal.foreign body. – Retropharyngeal abscess. D. Metabolic. – Hypocalcemic tetany complicating rickets. II. Chronic A. Congenital: – Congenital laryngeal stridor. – Congenital laryngeal stenosis – Congenital web, papilloma, or hemangioma. B. Acquired – Foreign body. – Tumor (rare). 1. ViralCroup: The most common cause of stridor in infancy and childhood. Etiology : Parainfluenza, adenovirus, respiratory syncytial virus and measles virus. Season: Early winter. Age: 3 months to 3 years. Clinical picture : Gradual onset starting by symptoms of upper respiratory tract infection for 1 -2 days followed by Harsh barking cough, Hoarseness of voice, inspiratory stridor worse by night. Fever is low grade. In severe cases, the condition progresses into continuous stridor, air hunger and cyanosis. The condition usually resolves within few days. Treatment: Most cases can be managed at home. Use of steam from a hot shower in a closed bathroom can improve the case. In severe cases (marked , cyanosis, irritability, marked intercostal retractions and disturbed sensorium), hospitalization is essential, I.V. fluids, humidified oxygen, nebulized epinephrine by inhalation and dexamethasone may improve the case. If no response, tracheostomy is life saving. . 2. Acute epiglottitis: Less common but more serious than viral croup. Etiology : Bacterial infection mainly hemophilus influenza type B. Clinical Picture: – Age: 3-7 years. – Onset: sudden, rapid death from acute respiratory obstruction may occur within few hours, – Typically the patient presents with acute onset of high fever (39-40°C), severe sore -throat, marked toxemia, dysphagia with drooling of saliva, weak voice or aphonia, and stridor. Examination of the patient with tongue depressor is dangerous as it may precipitate suffocation. It must be done in the operating theater in the presence of anaesthesia specialist to do an emergency tracheostomy if needed. Investigations. 1. Lateral x-ray on the neck: narrowing in the supra-glottic region. 2. Direct laryngoscopy (in the operating theater): the epiglottis appears red and swollen. Management – Hospitalization. – Endotracheal tube is introduced – ; Ampiciilin + chloramphenicol, I.V. for 10 days. 3. Diphtheritic fatyngitis: – Usually secondary to pharyngeal diphtheria, ft is characterized by gradual onset, mild sore throat, low grade fever, grayish membrane formed on tonsils extending into the larynx, enlarged cervical lymph nods, marked toxemia, in addition to hoarseness of voice, barking cough and marked stridor. – By time, the child becomes very ill, death from laryngeal obstruction may follow unless tracheostomy is performed + administration of Penicillin and Antitoxin. 4. Hypocafcemia stridor: – The patient is rachitic. Tetany is usually precipitated by infection. The stridor is usually precipitated by crying or any irritation to the child (revise tetany). 5. Acute spasmodic laryngitis (mid-night croup): – Acute, self limited and usually recurrent laryngeal spasm. Etiology is unknown, may be due to emotional disturbances. Clinical picture: – Sudden onset at midnight of barking cough, hoarseness of voice, stridor, and respiratory distress with tachycardia. The attack usually subsides alter 1-4 hours, It-may recur in the same night or the next 1-2 nights. Treatment: – Humidification (steam from hot shower) is usually sufficient. 6. Congenital larytigeal stridor: It is caused by laryngomalacia or tracheomalacia. Stridor occurs immediately at or few days after birth. Clinical picture: – More common in boys. – Noisy respiration and stridor starts immediately or few days after birth. Diagnosis : – Diagnosis is easy by direct laryngoscopy. – The condition usually improves spontaneously by the age of 1 year. 7. Congenital web or papilloma: need surgical removal. 8. Angioneurotic edema: may be associated with other evidences of allergy as urticarial skin rash. It improves by epinephrine or hydrocortisone. 9. Laryngeal foreign body : very important in pediatric ages. The obstruction is mechanical followed by inflammation.

Bronchitis

Bronchitis is inflammation of the large airways that branch off the trachea (bronchi), usually caused by infection but sometimes caused by irritation from inhaling gases, smoke, dust particles, or some types of pollution.  Acute bronchitis is usually caused by viral infections.  Symptoms of the common cold that are followed by a cough usually indicate acute bronchitis.  The diagnosis is made based primarily on symptoms.  Most treatments, such as cough suppressants and drugs to reduce fever, are used to make the person more comfortable until the episode ends.  Antibiotics are almost never needed. Symptoms lasting up to 90 days are usually classified as acute bronchitis; symptoms lasting longer, sometimes for months or years, are usually classified as chronic bronchitis. This chapter discusses acute bronchitis only. Causes Acute bronchitis can be caused by infection or by exposure to irritants. Understanding Bronchitis

In bronchitis, areas of the bronchial wall become inflamed and swollen, and mucus increases. As a result, the air passageway is narrowed.

Infectious Bronchitis: Infectious bronchitis occurs most often during the winter and is most often caused by viruses. Viral bronchitis may be caused by a number of common viruses, including the influenza virus. Even after a viral infection has resolved, the irritation it causes can continue to cause symptoms for weeks. Infectious bronchitis may also be caused by bacteria. Often bacterial bronchitis follows a viral upper respiratory infection. Acute bronchitis is more likely to be caused by bacteria in people who smoke. Mycoplasma pneumoniae and Chlamydia pneumoniae are the bacteria that often cause acute bronchitis in young adults. In rare cases, Bordetella pertussis infection (whooping cough) may cause acute bronchitis. Smokers and people who have chronic lung diseases may have repeated attacks of acute bronchitis. These episodes may be caused by bacteria, viruses, irritation from inhaling smoke, or a combination of factors. Undernutrition increases the risk of upper respiratory tract infections and subsequent acute bronchitis, especially in children and older people. Chronic sinus infections, bronchiectasis (see Bronchiectasis), and allergies also increase the risk of repeated episodes of acute bronchitis. Children with enlarged tonsils and adenoids may have repeated episodes of bronchitis.

Irritative Bronchitis: Irritative bronchitis (also called industrial or environmental bronchitis) may be caused by exposure to various mineral and dusts as well as cigarette smoke and smog. Exposure to fumes from strong acids, ammonia, some organic solvents, chlorine, hydrogen sulfide, dioxide, and bromine can also cause irritative bronchitis. Symptoms Infectious bronchitis generally begins with the symptoms of a common cold: runny nose, sore throat, fatigue, and chilliness. Back and muscle aches together with a slight fever (100° to 101° F, or 37.5° to 38° Celsius [C]) may be present, particularly if the infection is due to influenza. The onset of cough (usually dry at first) signals the beginning of acute bronchitis. With viral bronchitis, small amounts of white mucus are often coughed up. This mucus often changes from white to green or yellow. The color change does not mean there is a bacterial infection. Color change means only that cells associated with inflammation have moved into the airway and are coloring the . With severe bronchitis, fever may be slightly higher at 101° to 102° F (38° to 39° C) and may last for 3 to 5 days, but higher fevers are unusual unless bronchitis is caused by influenza. Cough is the last symptom to subside and often takes several weeks or even longer to do so. Viruses can damage the epithelial cells lining the bronchi, and the body needs time to repair the damage. Airway hyperreactivity, which is a short-term narrowing of the airways with impairment or limitation of the amount of air flowing into and out of the lungs, is common with acute bronchitis. The impairment of airflow may be triggered by common exposures, such as inhaling mild irritants (for example, perfume, strong odors, or exhaust fumes) or cold air. If the impairment of airflow is severe, the person may be short of breath. Wheezing, especially after coughing, is common.

Complications: Serious complications, such as acute respiratory failure or pneumonia, usually occur only in people who have advanced underlying chronic lung disease, such as chronic obstructive pulmonary disease, who are older, or who have problems with immune defenses.

Diagnosis Doctors usually make a diagnosis of bronchitis based on the symptoms. Fevers that are high or prolonged or both could indicate the presence of pneumonia. Doctors may hear wheezing during the physical examination. A chest x-ray is sometimes done to exclude pneumonia, mainly when doctors hear wheezing or congestion in the lungs or when the person is short of breath. A sample taken from the throat can be used to detect influenza. Sputum is generally only examined if doctors find evidence of pneumonia on a chest x-ray or during the examination. If a cough persists for more than 2 months, a chest x-ray is done to exclude an underlying lung disease, such as lung .

Treatment Cough medicines can be used to suppress a dry, disturbing cough, particularly when it interferes with sleep. However, a cough that produces a lot of sputum usually should not be suppressed. Expectorants may help to thin secretions and make them easier to cough up, but whether this is helpful is not clear. Сhildren should take only acetaminophen or ibuprofen, not aspirin. People with acute bronchitis, especially those who have a fever, should drink plenty of fluid. Antibiotics are not used to treat bronchitis except for people whose infection is caused by Bordetella pertussis or for some people with chronic obstructive pulmonary disease. Antibiotics do not help people with viral bronchitis. However, if influenza is the suspected cause, treatment with an may be helpful if given within 48 hours of onset of symptoms. In children, very mild symptoms of limited airflow can be helped with cool-mist humidifiers or steam vaporizers. In more severely affected children and adults who are wheezing, inhaled , which widen the bronchi, can be used to open the airways and reduce wheezing. , usually given by means of a metered-dose inhaler, are also sometimes used to diminish cough and inflammation and hyperreactivity of the airways, especially when the cough persists after the infection has resolved.

Bronchiolitis Bronchiolitis is an acute wheezing-associated illness, which occurs in early life, preceded by signs and symptoms of an upper respiratory infection. Infants may have a single episode or may have multiple occurrences in the first year of life.

I. Epidemiology

 Bronchiolitis occurs most frequently from early November and continues through April.  Bronchiolitis is most serious in infants who are less than one year old, especially those 1-3 months old. Infants at risk include those who are raised in crowded living conditions, who are passively exposed to tobacco smoke, and who are not breast-fed.

II. Pathophysiology  Respiratory syncytial virus (RSV) is the leading cause of bronchiolitis in infants and young children,accounting for 50% of cases of bronchiolitis requiring hospitalization.

 Infants born prematurely, or with chronic lung disease (CLD), immunodeficiency or congenital heart disease are at especially high risk for severe RSV illness.  RSV is transmitted bycontact with nasal secretions. Shedding of virus occurs 1 to 2 days before symptoms occur, and for 1 to 2 weeks afterwards. Symptoms usually last an average of 5 days.  Parainfluenza viruses are the second most frequent cause of bronchiolitis. They cause illness during autumn and spring, before and after outbreaks of RSV. Influenza Avirus,adenovirus, rhinovirus and Mycoplasma pneumonia can all cause bronchiolitis.Rhinovirus and mycoplasma pneumonia cause wheezing-associated respiratory illness in older children, while parainfluenza virus and RSV can cause wheezing at any age.

III. Clinical evaluation of bronchiolitis  Symptoms of RSV may range from those of a mild cold to severe bronchiolitis or pneumonia. RSV infection frequently begins with nasal discharge, pharyngitis, and cough. Hoarseness or laryngitis is not common. Fever occurs in most young children, with temperatures ranging from 38°/C to 40°/C (100.4°/F to 104°/F).  Hyperresonance of the chest wall may be present, and wheezing can be heard in most infants without auscultation. The wheezing sound is harsh and low in pitch, although severely affected infants maynot have detectable wheezing. Fine “” are usually heard on inspiration. Substernal and intercostal retractions are often noted.  Cyanosis of the oral mucosa and nail beds may occur in severely ill infants.Restlessness and hyperinflation of the chest wall are signs of impending respiratory failure.

IV. Diagnosis  Infants with bronchiolitis present symptoms of an upper respiratoryillness for several days and wheezing during the peak RSV season.  Chest radiographytypicallyshows hyperexpansion and diffuse interstitial pneumonitis. Consolidation is noted in about 25% of children, most commonly in the right upper or middle lobe.  Oxygen saturation values of <95% suggest the need for hospitalization.  Arterial blood gases should be obtained to assess the severity of respiratory compromise. Carbon dioxide levels are commonly in the 30-35 mm Hg range. Respiratory failure is suggested by CO2 values of 45-55 mm Hg. Oxygen tension below 66 mm Hg indicates severe disease.  White blood cell count may be normal or elevated slightly, and the differential count may show neutrophilia.  Enzyme-linked immunosorbent assays (ELISA) of nasal washings for RSV are highly sensitive and specific.

V. Management  Outpatient management of bronchiolitisis appropriate for infants with mild disease.  Crit eria for hospit alizatio n: 1. History of prematurity (especially less than 34 weeks) 2. Congenital heart disease 3. Other underlying lung disease 4. Low initial oxygen saturation suggestive of respiratory failure (O2 saturation <95%, with a toxic, distressed appearance) 5. Age <3 months 6. Dehydrated infant who is not feeding well 7. Unreliable parents  Before hospitalization, infants should receive an aerosolized beta-adrenergic agent. A few infants will respond tothis therapy and avoid hospitalization. If the response is good, the infant can be sent home, and oral albuterol continued.  Hospitalized infants should receive hydration and ambient oxygen to maintain an oxygen saturation >92-93% by pulse oximetry.  Treatment of bronchiolitis in the hospital 1. Racemic epinephrine by inhalation may be administered as a therapeutic trial. It should be continued if an improvement in the respiratory status is noted. Racemic epinephrine is administered as 0.5 mL of a 2.25% solution, diluted with 3.5 mL of saline (1:8) by nebulization. It is given every 20-30 minutes for severe croup, and it is given every 4-6 hours for moderate croup. 2. Ribavirin, an antiviral agent, produces modest improvement in clinical illness and oxygenation. Ribavirin is helpful in severely ill or high-risk patients. The dosage is 2 gm (diluted to 60 mg/mL) aerosolized over 2 hours tid for 3-5 days using an oxygen hood. Treatment with ribavirin combined with RSV immune globulin administered either parenterally or by aerosol is more effective than therapy with either agent alone. use in the treatment of bronchiolitis is not recommended.

Nebulizer

In medicine, a nebulizer is a device used to administer medication to people in the form of a mist inhaled into the lungs. It is commonly used in treating cystic fibrosis, , and other respiratory diseases.

There are different types of nebulizer, although the most common are the jet nebulizers, which are also called "atomizers". Jet nebulizers are connected by tubing to a compressed air source that causes air or oxygen to blast at high velocity through a liquid medicine to turn it into an aerosol, which is then inhaled by the patient.

As a general rule, doctors most commonly prescribe metered-dose inhalers for their patients, largely because these are more convenient and portable than nebulizers. However, jet nebulizers are commonly used in hospital settings for patients who have difficulty using inhalers, such as in serious cases of respiratory disease, or severe asthma attacks.

Newer, compact electronic nebulizers are also available. These nebulizers, such as the Pari eFlow, the Respironics i-Neb, the Omron MicroAir series, the Beurer Nebulizer IH30, and the Aerogen Aeroneb, use vibration of membranes or meshes to produce the aerosol and are more portable since they do not need the compressed air source that accompanies jet nebulizers. However, electronic nebulizers are more expensive. Piezoelectric nebulizers are used in electronic cigarettes.

Nebulizers accept their medicine in the form of a liquid solution, which is often loaded into the device upon use. Bronchodilators such as salbutamol (albuterol USAN) are often used and sometimes additionally ipratropium. Corticosteroids are also used. The reason they are inhaled instead of ingested is usually to target their effect to the respiratory tract, which speeds onset of action of the medicine and reduces side effects compared to other means of delivering these medicines.

Usually, the aerosolized medicine is inhaled through a tube-like mouthpiece, similar to that of an inhaler. The mouthpiece, however, is sometimes replaced with a face mask, similar to that used for inhaled anaesthesia, for ease of use with young children or the elderly, although mouthpieces are preferable if patients are able to use them since facemasks result in reduced lung delivery because of aerosol losses in the nose.

After use with corticosteroid, it is theoretically possible for patients to develop a yeast infection in the mouth (thrush) or hoarseness of voice (dysphonia), although these conditions are clinically very rare. To avoid these adverse effects, some clinicians suggest that the person who used the nebulizer should rinse his or her mouth. This is not true for bronchodilators; however, patients may still wish to rinse their mouths due to the unpleasant taste of some bronchodilating drugs.

Home Nebulizer Therapy

What is a nebulizer? A nebulizer changes liquid medicine into fine droplets (in aerosol or mist form) that are inhaled through a mouthpiece or mask. Nebulizers can be used to deliver (airway- opening) medicines such as albuterol (Ventolin®, Proventil® or Airet®) or ipratropium bromide (Atrovent®).

A nebulizer may be used instead of a metered dose inhaler (MDI). It is powered by a compressed air machine and plugs into an electrical outlet. Portable nebulizers, powered by an internal battery or cigarette lighter, are available for individuals requiring treatments away from home.

Nebulizer care guidelines Your home care company will show you how to use the nebulizer. You will need the following supplies to give the nebulizer treatment:

• Air compressor • Nebulizer cup • Mask or mouthpiece • Clean eye droppers or other measuring devices to dispense the medication

Treatment procedure

1. Place the air compressor on a sturdy surface that will support its weight. Plug the cord from the compressor into a properly grounded (three prong) electrical outlet.

2. Wash your hands with soap and warm water, and dry completely with a clean towel.

3. Carefully measure the medicine exactly as you have been instructed. Use a separate, clean measuring device (eyedropper or syringe) for each medicine.

4. Remove the top part of the nebulizer cup, as shown to the left.

5. Place your medicine in the bottom of the nebulizer cup, as shown to the right.

6. Attach the top portion of the nebulizer cup and connect the mouthpiece or face mask to the cup.

7. Connect the tubing to both the aerosol compressor and nebulizer cup.

8. Turn on the compressor with the on/off switch. Once you turn on the compressor, you should see a light mist coming from the back of the tube opposite the mouthpiece as shown to the left.

9. Sit up straight on a comfortable chair.

10. If you are using a mask, position it comfortably and securely on your face as shown to the right.

11. If you are using a mouth piece, place it between your teeth and seal your lips around it as shown to the left.. 12. Take slow, deep breaths through your mouth. If possible, hold each breath for two to three seconds before breathing out. This allows the medication to settle into the airways.

13. Continue the treatment until the medication is gone (about seven to 10 minutes).

14. If you become dizzy or feel "jittery," stop the treatment and rest for about five minutes. Then continue the treatment, but try to breathe more slowly. If these symptoms continue with future treatments, inform your health care provider.

15. Turn the compressor off.

16. Take several deep breaths and cough. Continue coughing and try to clear any secretions you might have in your lungs. Cough the secretions into a tissue and dispose of it properly.

17. Wash your hands with warm water and soap, and dry them with a clean towel.

Care of nebulizer Cleaning and disinfecting your equipment is simple, yet very important. Cleaning should be done in a dust- and smoke-free area away from open windows. Here is how to clean your equipment:

1. After each treatment, rinse the nebulizer cup with warm water, shake off excess water and let it air dry.

2. At the end of each day, the nebulizer cup, mask, or mouthpiece should be washed in warm, soapy water using a mild detergent, rinsed thoroughly, and allowed to air dry.

Note: There is no need to clean the tubing that connects the nebulizer to the air compressor. Do not put these parts in the dishwasher.

3. Every third day, after washing your equipment, disinfect the equipment using a vinegar/water solution or the disinfectant solution your supplier suggests.

To use the vinegar solution, mix 1/2 cup white vinegar with 1-1/2 cups of water. Soak the equipment for 30 minutes and rinse well under a steady stream of water. Shake off the excess water and allow to air dry on a paper towel. Always allow the equipment to completely dry before storing in a plastic, zipper storage bag.

Compressor care

1. Cover the compressor with a clean cloth when not in use. Keep it clean by wiping it with a clean, damp cloth as needed. 2. Do not put the air compressor on the floor either for treatments or for storage. 3. Check the air compressor's filter as directed. Replace or clean according to the directions from your equipment supplier. 4. Always have an extra nebulizer cup and mask or mouthpiece in case you need it. 5. Store your medicines in a cool, dry place. Check them often. If they have changed color or formed crystals, throw them away and replace them with new ones. 6. All equipment for your nebulizer therapy can be obtained through your equipment supplier.

6. Self-control materials: 1. Child, 5 years, is ill the second day. He has weakness, high t to 37,3 С°, spoiling of appetite, a cough is frequent, moist, considerable excretions from to the nose. General state: BR 20 per minute, vesicular resonance during percussion, moist rales is hearkened from two sides, after a cough character of rales changes. It is diagnosed AVRI, bronchitis. What type of treatment is most faithful? A sulfanilamidi B aminiglicosidi C antihistaminic D Ambrocsol + vitamins E cefalosporines 2. In 4 years old boy on a background the increase of temperature to 37,5 there is a cough with viscoussputum, dry rales are under lungs. What preparation is offered a patient for dilution of sputum: A. Acetylcysteinum B. Root an althaea C. Root of glyсуrrhiza D. Mukaltin E. Natriya of bensoati 3. A child 8 months was in-patient to the hospital with complaints about an unproductive, intensive cough during 2 days, wheezing breathe, increase of T° of body to 37,8°. Clinical picture: periopaly cyanosys; emphysematic form of chest; many different rales during auscultation. An obstructive bronchitis was diagnosed. What therapy will be basis? A Broncholitics B Prednisolonum C Antibiotics D Mukolitiki E Enzymes 4. Child of 1 year had acute ill with higt t - 39,2°С, repetition vomiting. uneasiness. Pharings with hyperemia and cianosis. Tremor of exstremites. Incriase of tendon reflex. Rigidnost of occipital muscles, doubtful symptom of Kerniga. Likvor: protein- 0,66 ‰, cytosis – separate cells. On the 4th day of disease state was a better, meningeal sign is disappear. Your diagnosis? A. Virus encephalitis B. Gripp C. Enterovirus infection, serosis meningitis D. Meningeal form of poliomyelitis E. Tuberkulosis meningitis 5. The child of 9 months, which was ill on AVRI, appeared suddenly tetanus, general cyanosis, loss of consciousness. General state: cloniko-tonic convulsions, skin of cyanosis, suds is on lips, the signs of rickets, changes from side of internal organs weren’t found out. What preparations must be entered at the first time? A. Preparations of calcium B. Sedative C. Vitamine D D. Anticonvulssion E. Glukokorticoid 6. At the boy 10 years old with haemophilia has sings of ARVI with high temperature. That cann’t preparations enter with a febrifuge purpose to this patient? A. Aspipin B. Analgin C. Panadol D. Pipolphen E. Papacetomol

7.A 2-year-old girl underwent a tracheotomy for severe upper airway obstruction due to cerebral palsy at 1 year of age. She has had two episodes of pneumonia over the past 6 months, the last of which occurred approximately 6 weeks ago. The parents are concerned about the pneumonias and request further evaluation. Of the following, the MOST appropriate next step is to A. administer oxygen at 1 L/min per tracheotomy B. perform a video swallowing study to test for aspiration C. perform frequent deep suctioning D. refer for tonsillectomy and adenoidectomy E. remove the tracheostomy tube

8.Last winter you diagnosed respiratory syncytial virus bronchiolitis in a 12-month-old boy. He had wheezing and required hospitalization for supportive care. His mother now asks what his chances are of wheezing this coming winter. Of the following, his chances of wheezing again are CLOSEST to A. 10% B. 25% C. 50% D. 75% E. 90%

9.A newborn has severe respiratory distress, cyanosis, and bradycardia shortly after birth. Physical examination reveals decreased breath sounds on the left, displaced heart sounds to the right, and a scaphoid abdomen. An arterial blood gas reveals: pH, 7.12; PCO2, 84 mm Hg; PO2, 48 mm Hg; base excess, -2.0 mEq/L. Of the following, the MOST appropriate initial treatment is A. bag-and-mask ventilation B. extracorporeal membrane oxygenation C. gastric tube suction D. administration E. surgical repair

10. You are examining a 5-year-old boy who is new to your practice. While taking the initial history, you discover that he has been hospitalized twice because of pneumonia. The last infection was sufficiently severe to require resection of the left lower lobe of his lung. The boy also has had problems with recurrent boils. The mother reports that she had a brother who died of a bacterial infection when he was 1 year of age. Of the following, the MOST likely cause of this child’s illness is A. chronic granulomatous disease B. DiGeorge syndrome C. severe combined immunodeficiency D. Wiskott-Aldrich syndrome E. X-linked agammaglobulinemia

7. Bibliography BASIC 1. Behrman Richard E., Robert M. Kliegman, Hal B. Jenson, eds. Nelson Texbook of Paediatrics. 2. Philadelfia: WB Saunders, 18th edition - 2014. – 994 р. – Р.638. 3. Buryak V.N., Makhmutov R.F., Poshehonova J.V. Children`s diseases. – Donetsk: Nord-Press, 2010. – 328 p. 4. Pediatry. Guidance aid / Ed. by O. Tiazhka – Kyiv: Medicine, 2007. 5. Рediatrics: textbook/ T.O. Kryuchko, O.Y. Abaturov, T.V. Kushnereva, at all. Edited by T.O. Kryuchko, O.Y. Abaturov. Kyiv: AUS Medicine Publishing, 2016. - 208 p.+ 2p. of color insert.

ADDITION 1. Upper Respiratory Tract Infection // Anne Meneghetti, MD, Assistant Professor of Medicine, Tufts University School of Medicine; Medical Broadcaster, Life, Love and Health Contributor Information and Disclosures Updated: Aug 12, 2009. 2. Illustrated Textbook of Paediatrics/ Ed.: T. Lissauer, G. Clayden.- Edinburg: Mosby, 2006.- 410 pp. 3. Forfar & Arneil’s Textbook of Pediatrics/ Ed.: N.Mc Intosh, P.J. Helms, R.L. Smyth, S. Logan.- Edinburg: Churchill Livingstone, 2008. - 1687 pp. 4. Further Reading Health Care Guideline: Diagnosis and Treatment of Respiratory Illness in Children and Adults INSTITUTE FOR CLINICAL SYSTEMS IMPROVEMENT Second Edition January 2008.

THEMATIC SITES: http://www.freebookcentre.net/medical_text_books_journals/pediatrics_online_texts_download. html http://pediatrics.aappublications.org http://www.generalpediatrics.com http://www.aap.org/en-us/Pages/Default.aspx http://hospitalpediatrics.org

The methodical instructions are compiled by Associate Prof Poda O.A.

Methodical instructions were discussed and approved at the meeting of department of Pediatrics №2 protocol № ___ of ______20____. Additions and changes were made “____” ______20____

Additions and changes were made “____” ______20____ Additions and changes were made “____” ______20____ Additions and changes were made “____” ______20____ Additions and changes were made “____” ______20____ Ministry of Health of Ukraine Ukrainian Medical Stomatological Academy

Approved at the meeting of department of Pediatrics №2 Minutes № 21 " 05 " 06 2020 Head of the department MD, Prof. Kryuchko T.O. ______

METHODICAL RECOMMENDATIONS FOR INDIVIDUAL WORK OF STUDENTS IN PREPARATION FOR THE PRACTICAL ( SEMINARS) CLASS

Academic discipline Pediatrics Module № 1 Substantial module № 1

Topic of lesson Pneumonia in children.

Course IV

Faculty Medical

Poltava 2020 1. Actuality of the subject. Pneumonia is a substantial cause of morbidity and mortality in childhood (particularly among children <5 yr of age) throughout the world, rivaling diarrhea as a cause of death in developing countries. With an estimated 146–159 million new episodes per yr in developing countries, pneumonia is estimated to cause approximately 4 million deaths among children worldwide. Currently, the incidence of community-acquired pneumonia in developed countries is estimated to be 0.026 episodes per child-year compared to 0.280 episodes per child-year in developing countries. 2. Concrete aims:  Define the patient's complaints.  Collect anamnesis (history) disease.  Be able to conduct the chest palpation, percussion and auscultation of lungs.  Know classification of pneumonia.  Identify the characteristic signs of pneumonia in different age of a child.  Make up a plan of investigation of a child with pneumonia  Be able to estimate general and biochemical blood analysis, nose and throat secretion investigation, pleural fluid investigation, to interpret functional lung investigations (spirography,oxygenometria), X-ray investigation.  Make a preliminary diagnosis of pneumonia.  Give a basic, pathogenetic and symptomatic therapy pneumonia.  To demonstrate the usage by medical specialist of moral-deontological principles in child's pulmonology. 3. Basic knowledge, skills, habits necessary for studying the subject (interdisciplinary integration). Names of previous disciplines Acquired skills and habits

1. Anatomy, histology Аnatomic and histological features of respiratory system at children

2. Physiology Peculiarities of the respiratory system and gaseous exchange in child organism in norm and pathology.

3. Biochemistry To give a clinical estimation of the changes of biochemical indexes of blood.

4. Propadeutic pediatrics Characteristic complaints, clinical syndromes at the diseases of the respiratory system. Ability to collect anamnesis, find out the special complaints, conduct the clinical examination of children with the diseases of the respiratory system.

5. Pharmacology To have knowledge about the basic groups of medical preparations which are used at treatment of pneumonia.

6. Public Health To use knowledge about the structure of medical help for child's population for the proper use of resources of the system of health protection in the plan of prophylaxis and treatment.

4. Tasks for independent work during preparation for the study: 4.1. The enumeration of the main terms, parameters, characteristics which the student is to assimilate while preparing for the class: Term Definition Cyanosis Rate of oxygen deficiency in the blood

Respiratory rate (bpm) Newborn - 40 - 60, 1 year 30 - 35, 5 years - 25, 10 years - 20, over 12 years - 20 - 16

Dyspnea Shortness of breath with a violation of its frequency,depth and rhythm

Types of breathlessness inspirator, expiratory, mixed

Types rales moist, wheezing

The degrees of respiratory insufficiency I, II, III

Spirography Method of graphic registration breath movements, indicating changes in lung volume

Types of Dry and exudative

Artificial Respiration breathing mouth to mouth, and mouth to nose

4.2. Theoretical questions for the class: 1. Etiology and pathogenesis of pneumonia in children. 2. Clinical manifestation of pneumonia according to pathogen and the age of a child. 3. Classification of pneumonia in children. 4. Laboratory diagnostics of pneumonia in children. 5. Evaluation of the functional state of lungs. 6. Principles of treatment of pneumonia in children (antimicrobial therapy, expectorants, antipyretics). 7. Complications of pneumonia in children: pleural effusion, empyema, pericarditis. 8. Classification of respiratory insufficiency. 9. Peculiarities of infusion therapy in acute pneumonia in children. 4.3. Practical tasks to be carried out in class: 1. Working with test tasks. 2. Work of students in chambers near the bed of sick children with pneumonia. 3. Interpretation of additional methods of examination. 4. Clinical analysis of model case. 5. Deciding of situation tasks. 6. Setting of treatment for every child with pneumonia. 5. Content of the topic: Pneumonia is an inflammation of the parenchyma of the lungs. Although most cases of pneumonia are caused by microorganisms, noninfectious causes include aspiration of food or gastric acid, foreign bodies, hydrocarbons, and lipoid substances, hypersensitivity reactions, and drug- or radiation-induced pneumonitis. The causes of lung infection in neonates and immunocompromised hosts are distinct from those affecting otherwise normal infants and children.

ETIOLOGY. The cause of pneumonia in an individual patient is often difficult to determine because direct culture of lung tissue is invasive and rarely performed. Cultures performed on specimens obtained from the upper respiratory tract or “sputum” generally do not accurately reflect the cause of lower respiratory tract infection. Using “state-of-the-art” diagnostic testing, a bacterial or viral cause of pneumonia can be identified in 40–80% of children with community- acquired pneumonia. Streptococcus pneumoniae (pneumococcus) is the most common bacterial pathogen, followed by Chlamydia pneumoniae and Mycoplasma pneumoniae. In addition to pneumococcus, other bacterial causes of pneumonia in previously healthy children in the United States include group A streptococcus (Streptococcus pyogenes) and Staphylococcus aureus. Streptococcus pneumoniae, Haemophilus influenzae, and Staphylococcus aureus are the major causes of hospitalization and death from pneumonia among children in developing countries, although in children with HIV infection, Mycobacterium tuberculosis, atypical mycobacterium, Salmonella, Escherichia coli, and Pneumocystis jirovecii Viral pathogens are a prominent cause of lower respiratory tract infections in infants and children <5 yr of age. Viruses are responsible for 45% of the episodes of pneumonia identified in hospitalized children in Dallas. Unlike bronchiolitis, for which the peak incidence is in the 1st yr of life, the highest frequency of viral pneumonia occurs between the ages of 2 and 3 yr, decreasing slowly thereafter. Of the respiratory viruses, influenza virus and respiratory syncytial virus (RSV) are the major pathogens, especially in children <3 yr of age. Other common viruses causing pneumonia include parainfluenza viruses, adenoviruses, rhinoviruses, and metapneumovirus. The age of the patient may help identify possible pathogens.

PATHOGENESIS. The lower respiratory tract is normally kept sterile by physiologic defense mechanisms, including the mucocil iary clearance, the properties of normal secretions such as secretory immunoglobulin A (IgA), and clearing of the airway by coughing. Immunologic defense mechanisms of the lung that limit invasion by pathogenic organisms include macrophages that are present in alveoli and bronchioles, secretory IgA, and other immunoglobulins.

Viral pneumonia usually results from spread of infection along the airways, accompanied by direct injury of the respiratory epithelium, resulting in airway obstruction from swelling, abnormal secretions, and cellular debris. The small caliber of airways in young infants makes them particularly susceptible to severe infection. Atelectasis, interstitial edema, and ventilation- perfusion mismatch causing significant often accompany airway obstruction. Viral infection of the respiratory tract can also predispose to secondary bacterial infection by disturbing normal host defense mechanisms, altering secretions, and modifying the bacterial flora.

When bacterial infection is established in the lung parenchyma, the pathologic process varies according to the invading organism. M. pneumoniae attaches to the respiratory epithelium, inhibits ciliary action, and leads to cellular destruction and an inflammatory response in the submucosa. As the infection progresses, sloughed cellular debris, inflammatory cells, and mucus cause airway obstruction, with spread of infection occurring along the bronchial tree, as it does in viral pneumonia.

S. pneumoniae produces local edema that aids in the proliferation of organisms and their spread into adjacent portions of lung, often resulting in the characteristic focal lobar involvement.

Group A streptococcus infection of the lower respiratory tract results in more diffuse infection with interstitial pneumonia. The pathology includes necrosis of tracheobronchial mucosa; formation of large amounts of exudate, edema, and local hemorrhage, with extension into the interalveolar septa; and involvement of lymphatic vessels and the increased likelihood of pleural involvement.

S. aureus pneumonia manifests in confluent bronchopneumonia, which is often unilateral and characterized by the presence of extensive areas of hemorrhagic necrosis and irregular areas of cavitation of the lung parenchyma, resulting in pneumatoceles, empyema, or, at times, bronchopulmonary fistulas.

CLINICAL MANIFESTATIONS. Viral and bacterial pneumonias are often preceded by several days of symptoms of an upper respiratory tract infection, typically rhinitis and cough. In viral pneu monia, fever is usually present; temperatures are generally lower than in bacterial pneumonia. Tachypnea is the most consistent clinical manifestation of pneumonia. Increased work of breathing accompanied by intercostal, subcostal, and suprasternal retractions, nasal flaring, and use of accessory muscles is common. Severe infection may be accompanied by cyanosis and respiratory fatigue, especially in infants. Auscultation of the chest may reveal crackles and wheezing, but it is often difficult to localize the source of these adventitious sounds in very young children with hyperresonant chests. It is often not possible to distinguish viral pneumonia clinically from disease caused by Mycoplasma and other bacterial pathogens. Bacterial pneumonia in adults and older children typically begins suddenly with a shaking chill followed by a high fever, cough, and . In older children and adolescents, a brief upper respiratory tract illness is followed by the abrupt onset of shaking chills and high fever accompanied by drowsiness with intermittent periods of restlessness; rapid respirations; a dry, hacking, unproductive cough; anxiety; and, occasionally, delirium. Circumoral cyanosis may be observed. Many children are noted to be splinting on the affected side to minimize pleuritic pain and improve ventilation; they may lie on their side with their knees drawn up to their chest.

Physical findings depend on the stage of pneumonia. Early in the course of illness, diminished breath sounds, scattered crackles, and rhonchi are commonly heard over the affected lung field. With the development of increasing consolidation or complications of pneumonia such as effusion, empyema, or pyopneumothorax, dullness on percussion is noted and breath sounds may be diminished. A lag in respiratory excursion often occurs on the affected side. Abdominal distention may be prominent because of gastric dilation from swallowed air or ileus. Abdominal pain is common in lower lobe pneumonia. The may seem enlarged because of downward displacement of the diaphragm secondary to hyperinflation of the lungs or superimposed congestive heart failure. Nuchal rigidity, in the absence of meningitis, may also be prominent, especially with involvement of the right upper lobe.

Symptoms described in adults with pneumococcal pneumonia may be noted in older children but are rarely observed in infants and young children, in whom the clinical pattern is considerably more variable. In infants, there may be a prodrome of upper respiratory tract infection and diminished appetite, leading to the abrupt onset of fever, restlessness, apprehension, and respiratory distress. These infants appear ill with respiratory distress manifested by grunting; nasal flaring; retractions of the supraclavicular, intercostal, and subcostal areas; tachypnea; tachycardia; air hunger; and often cyanosis. Results of physical examination may be misleading, particularly in young infants, with meager findings disproportionate to the degree of tachypnea. Some infants with bacterial pneumonia may have associated gastrointestinal disturbances characterized by vomiting, anorexia, diarrhea, and abdominal distention secondary to a paralytic ileus. Rapid progression of symptoms is characteristic in the most severe cases of bacterial pneumonia.

Abnormal Respiratory Rates by Age Age Abnormal

<2 months >60 bpm 2-12 months old >50 bpm >1 year old >40 bpm

DIAGNOSIS. The chest radiograph confirms the diagnosis of pneumonia and may indicate a complication such as a pleural effusion or empyema. Viral pneumonia is usually characterized by hyperinflation with bilateral interstitial infiltrates and peribronchial cuffing. Confluent lobar consolidation is typically seen with pneumococcal pneumonia. The radiographic appearance alone is not diagnostic and other clinical features must be considered. Repeat chest x-rays are not required for proof of cure for patients with uncomplicated pneumonia. The peripheral white blood cell (WBC) count can be useful in differentiating viral from bacterial pneumonia. In viral pneumonia, the WBC count can be normal or elevated but is usually not higher than 20,000/mm3, with a lymphocyte predominance. Bacterial pneumonia (occasionally, adenovirus pneumonia) is often associated with an elevated WBC count in the range of 15,000- 40,000/mm3 and a predominance of granulocytes. A large pleural effusion, lobar consolidation, and a high fever at the onset of the illness are also suggestive of a bacterial etiology. Atypical pneumonia due to C. pneumoniae or M. pneumoniae is difficult to distinguish from pneumococcal pneumonia by x-ray and other labs, and although pneumococcal pneumonia is associated with a higher WBC count, erythrocyte sedimentation rate (ESR), and C–reactive protein (CRP), there is considerable overlap. The definitive diagnosis of a viral infection rests on the isolation of a virus or detection of the viral genome or antigen in respiratory tract secretions. Growth of respiratory viruses in tissue culture usually requires 5–10 days. Reliable DNA or RNA tests for the rapid detection of RSV, parainfluenza, influenza, and adenoviruses are available and accurate. Serologic techniques can also be used to diagnose a recent respiratory viral infection but generally require testing of acute and convalescent serum samples for a rise in antibodies to a specific viral agent. This diagnostic technique is laborious, slow, and not generally clinically useful because the infection usually resolves by the time it is confirmed serologically. Serologic testing may be valuable as an epidemiologic tool to define the incidence and prevalence of the various respiratory viral pathogens. The definitive diagnosis of a bacterial infection requires isolation of an organism from the blood, pleural fluid, or lung. Culture of sputum is of little value in the diagnosis of pneumonia in young children. Blood cultures are positive in only 10% of children with pneumococcal pneumonia. In M. pneumoniae infections, cold agglutinins at titers >1 : 64 are found in the blood in ≈50% of patients. Cold agglutinins are nonspecific, however, because other pathogens such as influenza viruses may also cause increases. Acute infection caused by M. pneumoniae can be diagnosed on the basis of a positive PCR test or seroconversion in an IgG assay. Serologic evidence such as the anti-streptolysin O (ASO) titer may be useful in the diagnosis of group A streptococcal pneumonia.

TREATMENT. Treatment of suspected bacterial pneumonia is based on the presumptive cause and the clinical appearance of the child. For mildly ill children who do not require hospitalization, amoxicillin is recommended. In communities with a high percentage of penicillin-resistant pneumococci, high doses of amoxicillin (80–90 mg/kg/24 hr) should be prescribed. Therapeutic alternatives include cefuroxime axetil or amoxicillin/clavulanate. For school-aged children and in those in whom infection with M. pneumoniae or C. pneumoniae (atypical pneumonias) is suggested, a macrolide such as azithromycin is an appropriate choice. In adolescents, a respiratory fluoroquinolone (levofloxacin, gatifloxacin, moxifloxacin, gemifloxacin) may be considered for atypical pneumonias. The empirical treatment of suspected bacterial pneumonia in a hospitalized child requires an approach based on the clinical manifestations at the time of presentation. Parenteral cefuroxime (150 mg/kg/24 hr), cefotaxime, or ceftriaxone is the mainstay of therapy when bacterial pneumonia is suggested. If clinical features suggest staphylococcal pneumonia (pneumatoceles, empyema), initial antimicrobial therapy should also include vancomycin or clindamycin.

If viral pneumonia is suspected, it is reasonable to withhold antibiotic therapy, especially for those patients who are mildly ill, have clinical evidence suggesting viral infection, and are in no respiratory distress. Up to 30% of patients with known viral infection may have coexisting bacterial pathogens. Therefore, if the decision is made to withhold antibiotic therapy based on presumptive diagnosis of a viral infection, deterioration in clinical status should signal the possibility of superimposed bacterial infection and antibiotic therapy should be initiated.

Factors Suggesting Need for Hospitalization of Children with Pneumonia:  Age <6 mo  Sickle cell anemia with acute chest syndrome  Multiple lobe involvement  Immunocompromised state  Toxic appearance  Severe respiratory distress  Requirement for supplemental oxygen  Dehydration  Vomiting  No response to appropriate oral antibiotic therapy Noncompliant parents

RESPONSE TO TREATMENT. Typically, patients with uncomplicated community-acquired bacterial pneumonia respond to therapy with improvement in clinical symptoms (fever, cough, tachypnea, chest pain) within 48– 96 hr of initiation of antibiotics. Radiographic evidence of improvement substantially lags behind clinical improvement. A number of factors must be considered when a patient does not improve on appropriate antibiotic therapy (slowly resolving pneumonia): (1) complications, such as empyema; (2) bacterial resistance; (3) nonbacterial etiologies such as viruses and aspiration of foreign bodies or food; (4) bronchial obstruction from endobronchial lesions, foreign body, or mucous plugs; (5) pre-existing diseases such as immunodeficiencies, ciliary dyskinesia, cystic fibrosis, pulmonary sequestration, or cystic adenomatoid malformation; and (6) other noninfectious causes (including bronchiolitis obliterans, hypersensitivity pneumonitis, eosinophilic pneumonia, aspiration, and Wegener granulomatosis). A repeat chest x- ray is the 1st step in determining the reason for delay in response to treatment.

COMPLICATIONS. Complications of pneumonia are usually the result of direct spread of bacterial infection within the thoracic cavity (pleural effusion, empyema, pericarditis) or bacteremia and hematologic spread. Meningitis, suppurative arthritis, and osteomyelitis are rare complications of hematologic spread of pneumococcal or H. influenzae type b infection. S. aureus, S. pneumoniae, and S. pyogenes are the most common causes of parapneumonic effusions and of empyema. The treatment of empyema is based on the stage (exudative, fibrinopurulent, organizing). Imaging studies including ultrasonography and CT are helpful in determining the stage of empyema. The mainstays of therapy include antibiotic therapy and drainage with tube thoracostomy. Additional approaches include the use of fibrinolytic therapy (urokinase, streptokinase, alteplase) and selected video-assisted thoracoscopy (VATS) to debride, lyse adhesions, and drain loculated areas of pus. Early diagnosis and intervention, particularly with VATS, may obviate the need for thoracotomy and open debridement.

DIFFERENTIAL DIAGNOSIS OF PNEUMONIA • Pulmonary infarction • Pulmonary/pleural TB • Pulmonary oedema (can be unilateral) • Pulmonary eosinophilia • Malignancy: bronchoalveolar cell carcinoma • Rare disorders: cryptogenic organising pneumonia/bronchiolitis obliterans organising pneumonia (COP/BOOP)

COMPLICATIONS OF PNEUMONIA • Para-pneumonic effusion-common • Empyema • Retention of sputum causing lobar collapse • Development of thromboembolic disease • Pneumothorax-particularly with Staph. aureus • Suppurative pneumonia/lung abscess-see below • ARDS, renal failure, multi-organ failure • Ectopic abscess formation (Staph. aureus) • Hepatitis, pericarditis, myocarditis, meningoencephalitis • Pyrexia due to drug hypersensitivity

6. Self-control materials: Question: 1 A 3-year-old boy presents to the emergency department with a 1-month history of fever, cough, and weight loss. His parents are Cambodian, although he was born in the United States. In talking with his parents through an interpreter, you learn that relatives from Cambodia recently visited the family. Of the following, the diagnostic test MOST likely to yield the cause of this child's illness is a A. blood culture B. Mycoplasma serology on serum C. nasopharyngeal swab for viral culture D. skin test with purified protein derivative E. sweat chloride test Question: 2 You are evaluating a 10-year-old boy for his fourth episode of right middle lobe pneumonia, as demonstrated by chest radiography. He has a chronic cough and occasionally . Upon reviewing the chart, you note that he has had poor weight gain since these episodes began 2 years ago. Of the following, the BEST test to evaluate this problem is A. arterial blood gas measurement B. bronchography C. pulmonary function testing D. sweat chloride test E. thin-section high-resolution computed tomography Question: 3 A 16-year-old boy who has myasthenia gravis presents with a cough that is productive of green sputum. He had Pseudomonas aeruginosa pneumonia 3 months ago. On physical examination, he has a temperature of 102.4°F (39.1°C), respiratory rate of 28 breaths/min, and weight of 60 kg. He has left basilar rales. Pulse oximetry shows oxygen saturation of 93%. Forced vital capacity is 500 mL. Of the following, the MOST appropriate initial management for this patient is A. administration of intravenous gentamicin B. arterial blood gas determination C. chest physical therapy D. emergent tracheostomy E. endotracheal intubation and mechanical ventilation Question: 4 A mother brings in her daughter 4 days after you have prescribed amoxicillin for an ear infection. The girl had presented initially with fever, rhinitis, and ear pain of 2 days' duration. This morning she developed a rash all over her body. Results of the physical examination are normal except for the presence of a diffuse fine macular rash. Of the following, your BEST next step is to A. change the prescription to azithromycin B. immediately refer the girl to an allergist C. inform the mother that the girl never can receive amoxicillin again D. inform the mother that the girl's rash is probably due to a virus E. prescribe an antihistamine to treat the girl's drug allergy Question: 5 A 3-year-old boy is new to your practice. He has a history of recurrent otitis media that has been treated with multiple antibiotics. His previous physician had told the family that he was allergic to the following antibiotics: penicillin, sulfamethoxazole-trimethoprim, azithromycin, clarithromycin, and clindamycin. His mother would like to have tests done to determine if he is still allergic to these antibiotics. The ONLY one of these antibiotics for which immunoglobulin E (IgE) testing is available is A. azithromycin B. clarithromycin C. clindamycin D. penicillin E. sulfamethoxazole-trimethoprim 7. Bibliography

BASIC 1. Behrman Richard E., Robert M. Kliegman, Hal B. Jenson, eds. Nelson Texbook of Paediatrics. Philadelfia: WB Saunders, 18th edition - 2012. – 994 р. – Р.638. 2. Рediatrics: textbook/ T.O. Kryuchko, O.Y. Abaturov, T.V. Kushnereva, at all. Edited by T.O. Kryuchko, O.Y. Abaturov. Kyiv: AUS Medicine Publishing, 2016. - 208 p.+ 2p. of color insert. 3. Forfar & Arneil’s Textbook of Pediatrics/ Ed.: N.Mc Intosh, P.J. Helms, R.L. Smyth, S. Logan.- Edinburg: Churchill Livingstone, 2008. - 1687 pp. 4. Pediatry. Guidance aid / Ed. by O. Tiazhka – Kyiv: Medicine, 2007. ADDITION 1. Illustrated Textbook of Paediatrics/ Ed.: T. Lissauer, G.Clayden.- Edinburg: Mosby, 2015.- 410 pp. 2. Forfar & Arneil’s Textbook of Pediatrics/ Ed.: N.Mc Intosh, P.J. Helms, R.L. Smyth, S. Logan.- Edinburg: Churchill Livingstone, 2008. - 1687 pp. Upper Respiratory Tract Infection // Anne Meneghetti, MD, Assistant Professor of Medicine, Tufts University School of Medicine; Medical Broadcaster, Life, Love and Health Contributor Information and Disclosures Updated: Aug 12, 2009. 1. Further Reading Health Care Guideline: Diagnosis and Treatment of Respiratory Illness in Children and Adults INSTITUTE FOR CLINICAL SYSTEMS IMPROVEMENT Second Edition January 2014. THEMATIC SITES: http://eu-acme.org/europeanurology/upload_articles/1-s2.0-S0302283814011816-main.pdf http://uroweb.org/wp-content/uploads/18_Urological-infections_LR.pdf http://www.freebookcentre.net/medical_text_books_journals/pediatrics_online_texts_download. html http://pediatrics.aappublications.org http://www.generalpediatrics.com http://www.aap.org/en-us/Pages/Default.aspx http://hospitalpediatrics.org

The methodical instructions are compiled by Associate Prof Poda O.A.

Methodical instructions were discussed and approved at the meeting of department of Pediatrics №2 protocol № ___ of ______20____. Additions and changes were made “____” ______20____ Additions and changes were made “____” ______20____ Additions and changes were made “____” ______20____ Additions and changes were made “____” ______20____ Additions and changes were made “____” ______20____ MINISTRY OF HEALTH OF UKRAINE UKRAINIAN MEDICAL STOMATOLOGICAL ACADEMY

“APPROVED” at the meeting of department of Pediatric № 2 “____05__” ______06______2020 Protocol № 21 Head of the department MD, Prof. Kryuchko T.O. ______

Methodical instructions for independent work of students during preparation to practical classes and in class

Academic discipline Pediatrics

Module # 2 Topic of the lesson Hereditary, congenital and chronic diseases bronchopulmonary system in children Course 4 Faculty Medical

Poltava 2020 1. Actuality of the subject. Congenital malformations - anomalies, in most cases lead to serious changes in the structure and function of an organ or tissue. Malformation of lung diagnosed in 1-3% of newborn deaths and about 1 / 5 - 1 / 6 patients with chronic bronchopulmonary pathology Recognition of these states is very difficult. They are often mistaken for common chronic nonspecific lung diseases. However, a clear definition of the true nature of the pathological process ensures the success of therapeutic interventions, improves prognosis.

2. Concrete aims: - Define the patient's complaints. - Collect anamnesis (history) disease. - Be able to conduct the chest palpation, percussion and auscultation of lungs. - Know classification of pneumonia. - Identify the characteristic signs of pneumonia in different age of a child. - .Defining the concept: the rule options norms developmental abnormalities, malformation of the anatomical and functional state of the lungs; - Know the classification of malformations of the respiratory system in children - .Identify the tactics of the patient with the evils associated with hypoplasia of bronchopulmonary structures; - Identify the tactics of the patient with advanced and limited developmental defects of the trachea and bronchi; - Identify the tactics of a patient with lung cysts, Kartagener syndrome, - Demonstrate the ability of medical records in the clinic childhood diseases; - . Planning a survey sick child with congenital anomalies (malformations of the respiratory system in children), - Conduct a differential diagnosis and set a preliminary clinical diagnosis of congenital malformations of the respiratory system in children. - To substantiate the indications for bronchographic study; - To chart a comprehensive treatment of the child for the type of malformation of the respiratory system, individual reactivity of the body of the child and the severity of the disease.

3. Basic knowledge, skills, habits necessary for studying the subject (interdisciplinary integration). Names of previous disciplines Acquired skills and habits 1. Anatomy, histology Аnatomic and histological features of respiratory system at children 2. Physiology Peculiarities of the respiratory system and gaseous exchange in child organism in norm and pathology. 3. Biochemistry To give a clinical estimation of the changes of biochemical indexes of blood. 4. Propadeutic pediatrics Characteristic complaints, clinical syndromes at the diseases of the respiratory system. Ability to collect anamnesis, find out the special complaints, conduct the clinical examination of children with the diseases of the respiratory system. 5. Pharmacology To have knowledge about the basic groups of medical preparations which are used at treatment of pneumonia.

6. Public Health To use knowledge about the structure of medical help for child's population for the proper use of resources of the system of health protection in the plan of prophylaxis and treatment.

4. Tasks for independent work during preparation for the study: 2 4.1. The enumeration of the main terms, parameters, characteristics which the student is to assimilate while preparing for the class:

Term Definition Cyanosis Rate of oxygen deficiency in the blood Respiratory rate (bpm) Newborn - 40 - 60, 1 year 30 - 35, 5 years - 25, 10 years - 20, over 12 years - 20 - 16 Dyspnea Shortness of breath with a violation of its frequency, depth and rhythm Types of breathlessness inspirator, expiratory, mixed Types rales Moist, wheezing The degrees of respiratory insufficiency I, II, III Spirography Method of graphic registration breath movements, indicating changes in lung volume Types of pleurisy Dry and exudative Artificial Respiration breathing mouth to mouth, and mouth to nose Term Definition Variants of the rules Indicates a slight deviation from accepted norms of the structure, have no clinical significance. Malformations This functional disorders and disease manifestations exist or may arise. Agenesis of the lung The absence of lung, together with the main bronchus. Aplasia of the lung The absence of lung tissue in the presence of rudimentary main bronchus. Hypoplasia of the lung Simultaneous hypoplasia of the bronchi and lung parenchyma. Traheobronhomegaliya TBM (Cindy rum Inborn excessive expansion of the trachea and major Mounier-Kuhn) bronchi Traheobronhomalyatsiya The states in which there is increased mobility of the walls of the trachea and bronchi, narrowing of the lumen during expiration and increased during inspiration. William’s & Campbell syndrome The cause of the disease is hypoplasia cartilaginous rings of bronchial 3-8 orders of magnitude. Diverkul trachea and bronchi Single or multiple protrusions wall of the trachea and bronchi of any etiology. Sequestration of the lung Lack of communication area of the lung with bronchial system and its blood supply from anomalous artery (or arteries), the waste directly from the thoracic or abdominal aorta or its major branches. Kartagener syndrome Malformations with the triad of symptoms: the reverse layout of, bronchiectasis and chronic sinusitis.

4.2. Theoretical questions for the class: 1. Etiology and pathogenesis of pneumonia in children. 2. Clinical manifestation of pneumonia according to pathogen and the age of a child. 3. Classification of pneumonia in children. 4. Laboratory diagnostics of pneumonia in children. 5. Evaluation of the functional state of lungs.

3 6. Principles of treatment of pneumonia in children (Antimicrobial Therapy, expectorants, antipyretics). 7. Complications of pneumonia in children: pleural effusion, empyema, pericarditis. 8. Classification of respiratory insufficiency. 9. Peculiarities of infusion therapy in acute pneumonia in children. 10. Definition and classification of congenital malformations of the respiratory system. 11. Clinic, differential diagnostics. Diagnosis and treatment agenetsia and aplasia of the lungs 12. Simple and cystic hypoplasia of the lung and its particles, clinic, differential diagnosis, the role of additional methods of investigation, treatment. 13. Congenital traheobronhomalyatsiya, Williams-Campbell syndrome - congenital bronhomalyatsiya, clinical differential diagnosis, additional methods of examination, especially of bronchoscopy, the role of spirography, immunological studies. 14. Diagnostic features of limited malformations wall of the trachea and bronchus (congenital tracheal stenosis, congenital lobar emphysema; diverticul of the trachea, and bronchus, tracheobronchial fistula. The role of allied professionals in the diagnosis and treatment of these deficiencies. 15. The role of congenital (true) cysts in the formation of chronic bronchopulmonary pathology in children. Acquired cysts "honeycomb lung", differential diagnosis, complications. The role of CT for the timely diagnosis of lung cysts. 16. Features of diagnostic pulmonary sequestration, indications for surgical intervention. 17. Kartagener's syndrome (a syndrome of the ciliary dyskinesia), a triad of symptoms, other manifestations of degradation of the ciliary epithelium and the presence of congenital malformations of other organs. 18. Differential diagnosis of chronic hereditary and congenital diseases of bronchopulmonary system (cystic fibrosis, idiopathic pulmonary hemosiderosis, primary ciliary dyskinesia syndrome, Wilm’s‒ Campbell, bromhomalyatsia, aplasia of the lungs, ά1-antitrypsin deficiency, bronchopulmonary dysplasia, pulmonary sequestration) in children. 19. Tactics of the patient with hereditary, congenital and chronic bronchopulmonary diseases and their complications in children. 20. Prevention of hereditary, congenital and chronic diseases of the bronchopulmonary system in children.

4.3. Practical tasks to be carried out in class:

1. Working with test tasks. 2. Work of students in chambers near the bed of sick children with pneumonia. 3. Interpretation of additional methods of examination. 4. Clinical analysis of model case. 5. Deciding of situation tasks. 6. Setting of treatment for every child with pneumonia. 5. Content of the topic:

Bronchopulmonary Dysplasia History:  Maternal use of antenatal steroids  Gestational age, birth weight, APGAR score  Initial resuscitative efforts, need for intubation, use of , duration in intubation, type of ventilation, duration of supplemental oxygen therapy, and other factors: These may have influenced the type and degree of lung injury.  Familial history of asthma, atopy, or other children with bronchopulmonary dysplasia  Social support structure  Any potentially exacerbating factors, such as exposure to smoking 4 Physical exam:  , including careful assessment of work of breathing both at rest and during activity Feeding and sleeping history, and a review of growth charts  Vitals including respiratory rate and pulse oximetry both at rest and with activity  Signs of pulmonary hypertension, including peripheral edema, , and venous distention Tests:  Changes on chest radiography include hyperinflation, emphysema, cyst formation, , fibrosis, and cardiovascular changes. Severity of these changes may help predict the severity of the disease.  Electrocardiogram: Often followed serially to assess for right ventricular hypertrophy  Echocardiogram: Often a useful adjunct to follow patients with right ventricular hypertrophy  Cardiac catheterization: Reserved for patients with evidence of pulmonary hypertension and cardiac dysfunction  Pulmonary function testing: Often used to follow patients and evaluate responsiveness to interventions  Blood gases: Useful in acute and chronic management of bronchopulmonary dysplasia to follow the degree of hypoxia and  Bronchoscopy, barium swallow, pH probe, and sleep studies may reveal underlying conditions contributing to pulmonary dysfunction. Differencial diagnosis:  Asthma  Bronchiolitis obliterans  Congenital heart disease  Cystic adenomatoid malformation  Cystic fibrosis  Idiopathic pulmonary fibrosis  Infections  Meconium aspiration syndrome  Recurrent aspiration General measures:  Infants with bronchopulmonary dysplasia may have increased caloric needs as much as 150 kcal/kg/d.  Premature and critically ill infants may be deficient in antioxidants. Supplementation has not yet been shown to affect outcomes. Medication: : – Used for treating pulmonary edema, often improving lung mechanics and gas exchange – Furosemide may have other benefits, including effects on prostaglandin synthesis, direct vasodilatation, and improved surfactant production. – Side effects from long-term furosemide therapy include azotemia, ototoxicity, abnormalities, excessive urinary calcium loss, osteopenia, and nephrocalcinosis. – Thiazide diuretics, usually used with a potassium-sparing such as spironolactone, are not as effective as furosemide. – Routine monitoring of electrolytes is recommended for patients on long-term diuretic therapy. – Electrolyte supplementation is often required with long-term diuretic usage. Bronchodilators:

5 – Inhaled β-agonists are effective treatment for reversible bronchospasm, though safety and efficacy of long-term use has yet to be established. – Albuterol is often the drug of choice, though longer-acting agents are often used as well. – Muscarinic antagonists may be useful adjuncts, especially in patients who are not significantly responsive to albuterol. Believed to work on large and medium-sized airways – Cromolyn, though not a bronchodilator, is often used for its anti-inflammatory effects and has a low side-effect profile. – Methylxanthines are often used in the treatment of apnea, have a mild diuretic effect, and help improve diaphragmatic contractility, making them potentially useful in bronchopulmonary dysplasia. Pulmonary vasodilators: – Supplemental oxygen is an effective vasodilator and remains a mainstay of treatment for infants with hypoxia. Steroids: – Steroid usage is controversial. – Increased risk for sepsis has probably been overstated. – Often used successfully in short regimens to wean ventilatory support and hasten extubation – No long-term benefits of steroid therapy have been demonstrated. – Inhaled steroids may provide anti-inflammatory effects without systemic side effects, making them attractive as both prevention and treatment. – Routine use in premature infants is an active area of investigation. – Linear growth retardation has been a concern. – Newer agents that can be nebulized are now available, improving drug delivery in small infants. Prognosis: ♦ Most survivors demonstrate slow, steady improvement. ♦ High death rate (17–47%) for patients with severe disease requiring prolonged mechanical ventilation ♦ No treatment modality has shown significant impact on the long-term outcome of chronic bronchopulmonary dysplasia. ♦ Survivors often have long-term pulmonary sequelae including hyperinflation, reactive airways, and exercise intolerance. ♦ Even older children and young adults who were thought to be asymptomatic can have abnormal responsiveness to exercise. ♦ Newer technologies, in particular high-frequency ventilation and exogenous surfactant, have improved survival rates for premature infants; however, reduction in the incidence and severity of bronchopulmonary dysplasia has been difficult to demonstrate. Complications: 1. Prolonged intubation may cause subglottic stenosis and tracheomalacia. 2. Pulmonary hypertension may occur as a result of vasculature damage and subsequent intimal proliferation, which may, in turn, produce right ventricular hypertrophy and, if severe enough, cor pulmonale. 3. Pulmonary edema often occurs secondary to increased pulmonary capillary permeability and increased pulmonary pressures. 4. Reactive airways, bronchospasm, and altered pulmonary mechanics owing to a poorly compliant lung may result in abnormal pulmonary function testing and increased work of breathing. 5. Malnutrition and growth failure may occur as a result of increased work of breathing and a subsequently high caloric expenditure.

6 6. Impaired lung defenses result in an increased susceptibility to infection, especially respiratory syncytial virus.

A tracheoesophageal fistula (TEF)(known as TOF in the UK) is an abnormal connection (fistula) between the and the trachea. TEF is a common congenital abnormality, but when occurring late in life is usually the sequela of surgical procedures such as a laryngectomy. Causes Congenital TEF can arise due to failed fusion of the tracheoesophageal ridges during the third week of embryological development. A fistula, from the Latin meaning ‘a pipe,’ is an abnormal connection running either between two tubes or between a tube and a surface. In tracheo-esophageal fistula it runs between the trachea and the esophagus. This connection may or may not have a central cavity; if it does, then food within the esophagus may pass into the trachea (and on to the lungs) or alternatively, air in the trachea may cross into the esophagus. TEF can also occur due to pressure necrosis by a tracheostomy tube in apposition to a nasogastric tube (NGT). Associations Babies with TEF or esophageal atresia are unable to feed properly. Once diagnosed, prompt surgery is required to allow the baby to take in food. Many TEF children have few problems after surgery, however a number develop feeding difficulties and chest problems. Some TEF babies are also born with other abnormalities, most commonly those described in VACTERL association - a group of anomalies which often occur together, including heart, kidney and limb deformities. 6% of babies with TEF also have a laryngeal cleft. Classification Fistulae between the trachea and esophagus in the newborn can be of diverse morphology and anatomical location, however, various pediatric surgical publications have attempted a classification system based on the below specified types. Not all types include both esophageal agenesis and tracheoesophageal fistula, but the most common types do. The letter codes are usually associated with the system used by Gross, while number codes are usually associated with Vogt. An additional type, "blind upper segment only" has been described, but this type is not usually included in most classifications. (For the purposes of this discussion, proximal esophagus indicates normal esophageal tissue arising normally from the pharynx, and distal esophagus indicates normal esophageal tissue emptying into the proximal stomach.) Clinical presentation Tracheoesophageal fistula is suggested in a newborn by copious salivation associated with , coughing, vomiting, and cyanosis coincident with the onset of feeding. A fistula may also be a the cause of polyhydramnios while in utero. Treatment It is surgically corrected, with resection of any fistula and anastomosis of any discontinuous segments. Surgical repair is associated with complications, including • Stricture, due to gastric acid erosion of the shortened esophagus. • Leak of contents at the point of anastomosis. • Recurrence of fistula. ESOPHAGEAL ATRESIA AND TRACHEOESOPHAGEAL FISTULA What are the esophagus and trachea? • Esophagus: tube that connects the mouth to the stomach • Trachea: "windpipe" • Atresia: absence of a normal opening

7 • Congenital: found at birth • Fistula: abnormal passage from a body organ to the body surface or between two internal body organs.

Types of esophageal atresia/ tracheoesophageal fistula. The most common type is the left-most diagram. In each diagram 1=esophagus (upper and lower portions) and 2=trachea Congenital esophageal atresia (EA) represents a failure of the esophagus to develop as a continuous passage. Instead, it ends as a blind pouch. Tracheoesophageal fistula (TEF) represents an abnormal opening between the trachea and esophagus. EA and TEF can occur separately or together. EA and TEF are diagnosed in the ICU at birth and treated immediately. How are Esophageal Atresia and Tracheoesophageal Fistula diagnosed and treated? The presence of EA is suspected in an infant with excessive salivation (drooling) and in a newborn with drooling that is frequently accompanied by choking, coughing and sneezing. When fed, these infants swallow normally but begin to cough and struggle as the fluid returns through the nose and mouth. The infant may become cyanotic (turn bluish due to lack of oxygen) and may stop breathing as the overflow of fluid from the blind pouch is aspirated (sucked into) the trachea. The cyanosis is a result of laryngospasm (a protective mechanism that the body has to prevent aspiration into the trachea). Over time respiratory distress will develop. If any of the above signs/symptoms are noticed, a catheter is gently passed into the esophagus to check for resistance. If resistance is noted, other studies will be done to confirm the diagnosis. A catheter can be inserted and will show up as white on a regular x-ray film to demonstrate the blind pouch ending. Sometimes a small amount of barium (chalk-like liquid) is placed through the mouth to diagnose the problems. Treatment of EA and TEF is surgery to repair the defect. If EA or TEF is suspected, all oral feedings are stopped and intravenous fluids are started. The infant will be positioned to help drain secretions and decrease the likelihood of aspiration. Babies with EA may sometimes have other problems. Studies will be done to look at the heart and spine. Sometimes studies are done to look at the kidneys. Surgery to fix EA is rarely an emergency. Once the baby is in condition for surgery, an incision is made on the side of the chest. The esophagus can usually be sewn together. Following surgery, the baby may be hospitalized for a variable length of time. Care for each infant is individualized. If you have any questions do not hesitate to ask a member of the pediatric surgery team.

Kartagener Syndrome Background Siewert first described the combination of situs inversus, chronic sinusitis, and bronchiectasis in 1904. However, Manes Kartagener1 first recognized this clinical triad as a distinct congenital syndrome in 1933. Because Kartagener described this syndrome in detail, it bears his name. Kartagener syndrome (KS) is inherited via an autosomal recessive pattern. Symptoms result from defective cilia motility.

Pathophysiology Camner and coworkers first suggested ciliary dyskinesia as the cause of Kartagener syndrome in 1975. They described 2 patients with Kartagener syndrome who had immotile cilia and immotile 8 spermatozoa. These patients had poor mucociliary clearance because the cilia that lined their upper airways were not functioning. Later, Afzelius discovered that bronchial mucosal biopsy specimens from patients with similar respiratory complaints showed cilia that appeared abnormal, were poorly mobile, and were missing dynein arms. In 1977, Eliasson and coworkers used the descriptive phrase immotile cilia syndrome to characterize male patients with sterility and chronic respiratory infections. In 1981, Rossman and coworkers coined the term primary ciliary dyskinesia (PCD) because some patients with Kartagener syndrome had cilia that were not immobile but exhibited an uncoordinated and inefficient movement pattern. Current nomenclature classifies all congenital ciliary disorders as primary ciliary dyskinesias in order to differentiate them from acquired types. Kartagener syndrome is part of the larger group of disorders referred to as primary ciliary dyskinesias. Approximately one half of patients with primary ciliary dyskinesia have situs inversus and, thus, are classified as having Kartagener syndrome. Afzelius proposed that normal ciliary beating is necessary for visceral rotation during embryonic development. In patients with primary ciliary dyskinesia, organ rotation occurs as a random event; therefore, half the patients have situs inversus and the other half have normal situs. Ciliated epithelium covers most areas of the upper respiratory tract, including the nasal mucosa, paranasal sinuses, middle ear, eustachian tube, and pharynx. The lower respiratory tract contains ciliated epithelium from the trachea to the respiratory bronchioles. Each ciliated cell gives rise to approximately 200 cilia that vary in length from 5-6 μm and decrease in size as the airway becomes smaller. The typical ciliary axoneme consists of 2 central microtubules surrounded by 9 microtubular doublets. Each doublet has an A subunit and a B subunit attached as a semicircle. A central sheath envelops the 2 central microtubules, which attach to the outer doublets by radial spokes. The outer doublets are interconnected by nexin links, and each A subunit is attached to 2 dynein arms that contain adenosine triphosphatase; one inner arm and one outer arm. The primary function of the central sheath, radial spokes, and nexin links is to maintain the structural integrity of the cilium, whereas the dynein arms are responsible for ciliary motion. The cilium is anchored at its base by cytoplasmic microtubules and a basal body comprised of a basal foot and rootlet. The orientation of the basal foot indicates the direction of the effective cilial stroke. Just above the base, the cilium is composed of microtubular triplets (previously doublets) without associated structures, but at the tip, only the B subunits remain. Cilia propel overlying mucus via a 2-part ciliary beat cycle. First, the power stroke occurs when a fully extended cilium moves perpendicular to the cell surface in an arclike manner. Then, the recovery stroke follows, in which the entire cilium bends and returns to its starting point near the cell surface. Once a cilium starts to move, the complete beat cycle is obligatory. The cycle is mediated by dynein arms from the A subunit that attach to the B subunit of the adjacent microtubule. Adenosine triphosphate is hydrolyzed by the dynein arms and the 9 microtubule doublets as they slide against each other. Patients with primary ciliary dyskinesia exhibit a wide range of defects in ciliary ultrastructure and motility, which ultimately impairs ciliary beating and mucociliary clearance. The most common defect, first described by Afzelius, is a reduction in the number of dynein arms, which decreases the ciliary beat frequency. Sturgess et al described how the radial spoke, which serves to translate outer microtubular sliding into cilial bending, was absent in some patients with primary ciliary dyskinesia. Cilia in other patients lacked central tubules; however, instead of the central tubules, an outer microtubular doublet transposed to the cell of the axoneme was present that displayed an abnormal 8+1 doublet-to-tubule pattern. Both the radial spoke and the transposed doublet defects impaired mucociliary clearance. Other ciliary defects include an abnormal basal cell apparatus with giant roots and double feet, cilia lacking all internal microtubular structures, and even cilia twice the normal length that beat in an uncoordinated undulating fashion. Pedersen7 compared the type of ultrastructural defect to ciliary motility and found that dynein defects caused hypomotility and microtubular defects (ie, caused asynchrony). He also found that normal ciliary ultrastructure occasionally was associated with hypermotility or inefficient ciliary trembling. 9 Some patients with clinical features of primary ciliary dyskinesia have a ciliary ultrastructure that appears normal, but their arrangement and beat direction is disoriented, which causes inefficient mucociliary transport. These findings illustrate the importance of analyzing ciliary motility and ultrastructure when considering a diagnosis of primary ciliary dyskinesia.

Frequency The frequency of Kartagener syndrome is 1 case per 32,000 live births. Situs inversus occurs randomly in half the patients with primary ciliary dyskinesia; therefore, for every patient with Kartagener syndrome, another patient has primary ciliary dyskinesia but not situs inversus.

Physical Kartagener syndrome is characterized by the clinical triad of chronic sinusitis, bronchiectasis, and situs inversus.

• Upper airway o Nose: Patients may exhibit chronic, thick, mucoid rhinorrhea from early in childhood. Examination usually reveals pale and swollen nasal mucosa, mucopurulent secretions, and an impaired of smell. Nasal polyps are recognized in 30% of affected individuals. o Sinuses: The recurrent chronic sinusitis typically produces sinus pressure headaches in the maxillary and periorbital region. Symptoms usually improve with antibiotic therapy but have a propensity for rapid recurrence. o Ears: Recurrent otitis media is a common manifestation of primary ciliary dyskinesia. Examination may reveal a retracted tympanic membrane with poor or absent mobility and a middle-ear effusion. Other associated otologic disorders may include tympanosclerosis, cholesteatoma, and keratosis obturans. • Lower respiratory tract o Chronic bronchitis and recurrent pneumonia are common conditions in patients with primary ciliary dyskinesia. Thus, upon physical examination of the patient's chest, increased tactile , rhonchi, crackles, and, occasionally, may be present. o Obstructive lung disease may be another component of Kartagener syndrome symptomatology. It probably results from elevated levels of local inflammatory mediators in a chronically irritated airway. Therefore, wheezing may occur. The lung examination may be normal during intercurrent periods when the airway is not actively inflamed. • Other features o Cardiovascular examination of a patient with KS demonstrates a point of maximal impulse, and the heart sounds are heard best on the right side of the chest. o Extremities may exhibit digital clubbing.

Differential Diagnoses ♦ Alpha1-Antitrypsin Deficiency ♦ Tracheobronchomegaly ♦ Immunosuppression ♦ Yellow nail syndrome ♦ Adenoid hyperplasia ♦ Allergic bronchopulmonary aspergillosis ♦ Bronchial obstruction ♦ Chronic aspiration ♦ Congenital cartilage deficiency ♦ Cystic fibrosis ♦ Idiopathic nasal polyposis ♦ Inhalation of toxic substances ♦ Postinfectious bronchiectasis ♦ Pulmonary sequestration ♦ Samter triad ♦ Severe atopy 10

Laboratory Studies • Semen analysis in postpubescent males may reveal abnormal sperm motility and ultrastructure.

Imaging Studies • Sinus radiographs (which largely have been supplanted by CT scans) typically demonstrate mucosal thickening, opacified sinus cavities, and hypoplastic frontal sinuses. • Chest radiographs may illustrate bronchial wall thickening as an early manifestation of chronic infection, hyperinflation, atelectasis, bronchiectasis, and situs inversus (in 50% of patients with primary ciliary dyskinesia). The presence of situs inversus strongly suggests Kartagener syndrome (KS). • Bronchiectasis occurs in the lower lobes in patients with Kartagener syndrome and immunoglobulin deficiency, while bronchiectasis predominantly occurs in the upper lobes of patients with cystic fibrosis. • High-resolution CT scan of the chest is the most sensitive modality for documenting early and subtle abnormalities within airways and pulmonary parenchyma when compared to routine chest radiographs. Consideration should be given to this imaging technique early in the presentation of primary ciliary dyskinesia (PCD) syndromes, when a chest radiograph may not be sensitive enough to identify disease processes or when another differential is being considered.

Other Tests • Screening tests include the saccharin test and the measurement of nasal and exhaled nitric oxide. o Saccharine test: Saccharin or another substance is placed in the nose, and the speed of transport into the nasopharynx is measured to calculate mucociliary clearance (used infrequently because of awkwardness and dubious reliability). o Nitric oxide: In patients with primary ciliary dyskinesia, exhaled and nasal nitric oxide is low. • Audiologic testing usually demonstrates a flat tympanogram and bilateral conductive hearing loss secondary to thick middle-ear effusion. • Pulmonary function studies o Spirometry often reveals an obstructive ventilatory defect with decreases in the ratio of forced expired volume in 1 second to forced vital capacity, reduced forced expired volume in 1 second, and a reduced forced expiratory flow of 25-75%. o Static lung volumes also may demonstrate hyperinflation. o The response to bronchodilators is variable in patients with primary ciliary dyskinesia.

Histologic Findings The mucosal biopsy specimen should be examined for ciliary movement using light microscopy. Light microscopic quantitation of ciliary beat frequency, coordination, and amplitude, although available in very few medical centers, can identify ciliary dyskinesia in patients with normal ultrastructure. Light microscopy alone offers a reliable and simple method of excluding PCD, but light microscopy and electron microscopy in combination provide a higher degree of accuracy. The specimen should be placed in glutaraldehyde and sent for electron microscopy, which is the criterion standard examination for the diagnosis of primary ciliary dyskinesia. Quantitative diagnostic criteria do not exist; however, ciliary ultrastructure is examined qualitatively for abnormalities in dynein arms (inner and outer), radial spokes, central sheaths, nexin links, and ciliary transposition and orientation. The most common ultrastructural defect is an absence or decrease in the number of inner or outer dynein arms. A radial spoke deficiency commonly appears with a dynein arm deficiency. Other ultrastructural abnormalities with nexin links, central sheaths, and ciliary transposition and orientation are considered nonspecific for primary ciliary dyskinesia because they can occur in healthy people and those with recurrent respiratory infections. Electron microscopic diagnosis of ciliary ultrastructure is expensive, time consuming, and described by some experts as inadequate. Patients with Kartagener syndrome also may have normal ultrastructure, which decreases the sensitivity of electron microscopy. Efforts have been undertaken to standardize the clinical criteria for the diagnosis of Kartagener syndrome. These criteria include dextrocardia, a ciliary beat frequency of less than 10 Hz/s, and a mean cross-section dynein arm count of less than 2. If the patient does not have dextrocardia, primary ciliary dyskinesia presents a much greater diagnostic challenge. Genetic testing ultimately may become the principal means of establishing this diagnosis.

Medical Care • The most common infectious organisms affecting children with primary ciliary dyskinesia (PCD) are Haemophilus influenza and Staphylococcus aureus. All primary ciliary dyskinesia patients should receive comprehensive immunizations, including the influenza A and pneumococcal . • Antibiotics, intravenous or oral and continuous or intermittent, are used to treat upper and lower airway infections. Although prophylactic antibiotics should be used with great caution in this era of emerging antibiotic resistance, children with primary ciliary dyskinesia are especially good candidates for long-term low-dose preventative antibiotics. • Obstructive lung disease, if present, should be treated with inhaled bronchodilators and aggressive pulmonary toilet. Mucolytics may be helpful. Anecdotal reports indicate that inhaled antibiotics, oral and inhaled corticosteroids, and recombinant human DNAse have been used, but no large studies support the use of these agents.

Surgical Care • Tympanostomy tubes are required to reduce conductive hearing loss and recurrent infections. o Many patients undergo repeated tympanostomy tube insertions, often complicated by chronic suppurative otitis media. o Chronic otorrhea may require special measures for aural hygiene, such as regular otomicroscopy, acetic acid irrigations, or culture-guided topical or systemic antibiotic therapy. o Because of anticipated long-term middle-ear disease, inserting tympanostomy tubes is the most sensible method of maintaining the myringotomy because the tube can be expected to stay in the tympanic membrane longer than routine grommets. • When sinus disease is refractory to medical management, functional endoscopic sinus surgery leads to transient improvement in upper and lower respiratory tract symptoms. The antiquated procedure of making a nasal antral window underneath the inferior turbinate may have a role in the management of primary ciliary dyskinesia because this procedure relies on gravitational rather than ciliary clearance of mucus.

Early intervention should be instituted with antibiotics directed at specific organisms identified by nasal secretions and/or expectorated sputum samples. Sensitivities of these samples should be obtained because resistant microorganisms can develop. Mucolytics may be helpful in specific individuals.

Antibiotics Used to treat acute or chronic infection or for prophylaxis against infection. Empiric antimicrobial therapy must be comprehensive and should cover all likely pathogens in the context of the clinical setting.

Prognosis Chronic childhood infections can be very debilitating, but the range and severity of clinical symptoms is wide. Fortunately, primary ciliary dyskinesia (PCD) and Kartagener syndrome (KS) usually become less problematic near the end of the patient's second decade, and many patients have near normal adult lives.

12 Question: 1 A 3-year-old boy presents to the emergency department with a 1-month history of fever, cough, and weight loss. His parents are Cambodian, although he was born in the United States. In talking with his parents through an interpreter, you learn that relatives from Cambodia recently visited the family. Of the following, the diagnostic test MOST likely to yield the cause of this child's illness is a A. blood culture B. Mycoplasma serology on serum C. nasopharyngeal swab for viral culture D. skin test with purified protein derivative E. sweat chloride test

Question: 2 You are evaluating a 10-year-old boy for his fourth episode of right middle lobe pneumonia, as demonstrated by chest radiography. He has a chronic cough and occasionally hemoptysis. Upon reviewing the chart, you note that he has had poor weight gain since these episodes began 2 years ago. Of the following, the BEST test to evaluate this problem is A. arterial blood gas measurement B. bronchography C. pulmonary function testing D. sweat chloride test E. thin-section high-resolution computed tomography

Question: 3 A 16-year-old boy who has myasthenia gravis presents with a cough that is productive of green sputum. He had Pseudomonas aeruginosa pneumonia 3 months ago. On physical examination, he has a temperature of 102.4°F (39.1°C), respiratory rate of 28 breaths/min, and weight of 60 kg. He has left basilar rales. Pulse oximetry shows oxygen saturation of 93%. Forced vital capacity is 500 mL. Of the following, the MOST appropriate initial management for this patient is A. administration of intravenous gentamicin B. arterial blood gas determination C. chest physical therapy D. emergent tracheostomy E. endotracheal intubation and mechanical ventilation

Question: 4 A mother brings in her daughter 4 days after you have prescribed amoxicillin for an ear infection. The girl had presented initially with fever, rhinitis, and ear pain of 2 days' duration. This morning she developed a rash all over her body. Results of the physical examination are normal except for the presence of a diffuse fine macular rash. Of the following, your BEST next step is to A. change the prescription to azithromycin B. immediately refer the girl to an allergist C. inform the mother that the girl never can receive amoxicillin again D. inform the mother that the girl's rash is probably due to a virus E. prescribe an antihistamine to treat the girl's drug allergy

Question: 5 A 3-year-old boy is new to your practice. He has a history of recurrent otitis media that has been treated with multiple antibiotics. His previous physician had told the family that he was allergic to the following antibiotics: penicillin, sulfamethoxazole-trimethoprim, azithromycin, clarithromycin, and clindamycin. His mother would like to have tests done to determine if he is still allergic to these antibiotics. 13 The ONLY one of these antibiotics for which immunoglobulin E (IgE) testing is available is A. azithromycin B. clarithromycin C. clindamycin D. penicillin E. sulfamethoxazole-trimethoprim

BIBLIOGRAPHY

BASIC 1. Behrman Richard E., Robert M. Kliegman, Hal B. Jenson, eds. Nelson Texbook of Paediatrics. Philadelfia: WB Saunders, 18th edition - 2012. – 994 р. – Р.638. 2. Рediatrics: textbook/ T.O. Kryuchko, O.Y. Abaturov, T.V. Kushnereva, at all. Edited by T.O. Kryuchko, O.Y. Abaturov. Kyiv: AUS Medicine Publishing, 2016. - 208 p.+ 2p. of color insert. 3. Forfar & Arneil’s Textbook of Pediatrics/ Ed.: N.Mc Intosh, P.J. Helms, R.L. Smyth, S. Logan.- Edinburg: Churchill Livingstone, 2008. - 1687 pp. 4. Pediatry. Guidance aid / Ed. by O. Tiazhka – Kyiv: Medicine, 2007.

ADDITION

1. Illustrated Textbook of Paediatrics/ Ed.: T. Lissauer, G.Clayden.- Edinburg: Mosby, 2015.- 410 pp. 2. Forfar & Arneil’s Textbook of Pediatrics/ Ed.: N.Mc Intosh, P.J. Helms, R.L. Smyth, S. Logan.- Edinburg: Churchill Livingstone, 2008. - 1687 pp. Upper Respiratory Tract Infection // Anne Meneghetti, MD, Assistant Professor of Medicine, Tufts University School of Medicine; Medical Broadcaster, Life, Love and Health Contributor Information and Disclosures Updated: Aug 12, 2009. 1. Further Reading Health Care Guideline: Diagnosis and Treatment of Respiratory Illness in Children and Adults INSTITUTE FOR CLINICAL SYSTEMS IMPROVEMENT Second Edition January 2014. THEMATIC SITES: http://eu-acme.org/europeanurology/upload_articles/1-s2.0-S0302283814011816-main.pdf http://uroweb.org/wp-content/uploads/18_Urological-infections_LR.pdf http://www.freebookcentre.net/medical_text_books_journals/pediatrics_online_texts_download.html http://pediatrics.aappublications.org http://www.generalpediatrics.com http://www.aap.org/en-us/Pages/Default.aspx http://hospitalpediatrics.org

The methodical instructions are compiled by Associate Prof Poda O.A.

Methodical instructions were discussed and approved at the meeting of department of Pediatrics №2 protocol № ___ of ______20____. Additions and changes were made “____” ______20____

Additions and changes were made “____” ______20____ Additions and changes were made “____” ______20____ Additions and changes were made “____” ______20____ Additions and changes were made “____” ______20____ 14 MINISTRY OF HEALTH OF UKRAINE UKRAINIAN MEDICAL STOMATOLOGICAL ACADEMY

“APPROVED” at the meeting of department of Pediatric №2 “__05__” __06______2020 Protocol № 21 Head of the department MD, Prof. Kryuchko T.O. ______

Methodical instructions for independent work of students during preparation to practical classes and in class

Academic discipline Pediatrics

Module # 3 Topic of the lesson Atopic dermatitis and allergic rhinitis in children. Course 4 Faculty Medical

Poltava 2020

1. Actuality of the subject. Atopic dermatitis (AD), often called eczema or atopic eczema, is a very common skin disease. The exact cause is not known, but AD results from a combination of family heredity and a variety of conditions in everyday life that trigger the red, itchy rash. Atopic dermatitis has been reported to affect 10 percent of children. In the United States alone, it is estimated that more than $364 million per year is spent on the treatment of childhood atopic dermatitis. Although the symptoms of atopic dermatitis resolve by adolescence in 50 percent of affected children, the condition can persist into adulthood. Poor prognostic features include a family history of the condition, early disseminated infantile disease, female gender and coexisting allergic rhinitis and asthma. 2. Concrete aims: 1. Define the patient's complaints. 2. Collect anamnesis (history) disease. 3. Be able to conduct the chest palpation, percussion and auscultation of lungs. 4. Know classification of athopic dermatitis and allergic rhinitis. 5. Identify the characteristic signs of athopic dermatitis and nettle-rash in different age of a child. 6. Make up a plan of investigation of a child with athopic dermatitis and nettle-rash. 7. Be able to estimate general and biochemical blood analysis. 8. Make a preliminary diagnosis of athopic dermatitis and nettle-rash. 9. Give basic, pathogenetic and symptomatic therapy athopic dermatitis. 10. To demonstrate the usage by medical specialist of moral-deontological principles in child's allergology.

3. Basic knowledge, skills, habits necessary for studying the subject (interdisciplinary integration).

Names of previous disciplines Acquired skills and habits 1. Anatomy, histology Аnatomic and histological features of skin in children

2. Physiology Peculiarities of the skin and exchange in child organism in norm and pathology. 3. Biochemistry To give a clinical estimation of the changes of biochemical indexes of blood. 4. Propadeutic pediatrics Characteristic complaints, clinical syndromes at the diseases of the respiratory system. Ability to collect anamnesis, find out the special complaints, conduct the clinical examination of children with the diseases of the upper respiratory system. 5. Pharmacology To have knowledge about the basic groups of medical preparations which are used at treatment of athopic dermatitis and allergic rhinitis.

6. Public Health To use knowledge about the structure of medical help for child's population for the proper use of resources of the system of health protection in the plan of prophylaxis and treatment.

4. Tasks for independent work during preparation for the study: 4.1. The enumeration of the main terms, parameters, characteristics which the student is to assimilate while preparing for the class:

Term Definition

2 Irritants Any of the substances outside the body that can cause burning, redness, itching or dryness of the skin. Itch (Latin: pruritus) is a sensation that causes the desire or reflex to scratch. Itch has resisted many attempts to classify it as any one type of sensory experience. Dyspnea Shortness of breath with a violation of its frequency, depth and rhythm. Xerosis cutis Is the medical term for dry skin. Pityriasis alba Is a common skin condition mostly occurring in children and usually seen as dry, fine-scaled, pale patches on the face. It is self- limiting and usually only requires use of creams. Nipple eczema Skin conditions such as eczema or dermatitis may occur on the nipple and areola. White dermatographism Linear blanching of (usually erythematous) skin of persons with atopic dermatitis in response to firm stroking with a blunt instrument.

4.2.Theoretical questions for the class: 1. What is atopic dermatitis?  How do you know if it’s atopic dermatitis?  When does it begin? Where is it located? 2. Does it run in families? 3. What are the causes of atopic dermatitis? 4. What are the trigger factors? 5. How can you avoid trigger factors? 6. Tell me about pathogenesis of atopic dermatitis? 7. What are there major and minor features do you know? 8. What differential diagnosis is god for children with atopic dermatitis? 9. What is it SCORAD? 10. What kind of medicines do you use in atopic dermatitis treatment?  bathing and ;  antihistamines;  antibiotics;  corticosteroids;  leukotriene inhibitors;  immunosuppressants. 4.3. Practical tasks to be carried out in class: 1. Working with test tasks. 2. Work of students in chambers near the bed of sick children with atopic dermatitis. 3. Interpretation of additional methods of examination. 4. Clinical analysis of model case. 5. Deciding of situation tasks. 6. Setting of treatment for every child with atopic dermatitis, allergic rhinitis and nettle-rash. The prevalence of children with atopic dermatitis and other atopic conditions, such as hay fever and asthma, has increased in the last decade, particularly in westernized countries. Oranje, of the University Hospital, Rotterdam, Netherlands, reported that 10% to 20% of all children in western populations have or will be affected. Atopic dermatitis predominantly affects infants and children, but the disease can persist throughout life. An increase in both indoor and outdoor pollution may have contributed to the increased prevalence. Parental smoking and the migration of populations to urban areas subject to smog and other forms of pollution are considered major influences. Increasing maternal age and the western life style have also come under scrutiny.

How do we know if it’s atopic dermatitis? 3 1. Time of onset. This type of eczema usually begins during the first year of life and almost always within the first five years. It’s seldom present at birth, but it often comes on after six weeks. Other rashes also can start at that time, so it may be confusing at first, but most rashes disappear within a few days to weeks. AD tends to persist. It may wax and wane, but it keeps coming back. 2. Itching. Atopic dermatitis also is a very itchy rash. Much of the skin damage comes from scratching and rubbing that the child cannot control. 3. The location of the rash can also help us recognize AD. In babies, the rash usually starts on the face or over elbows and knees, places that are easy to scratch and rub. It may spread to involve all areas of the body, although the moist diaper region is often protected. Later in childhood the rash is typically in the elbow and knee folds. Sometimes it only affects the hands, and at least 70% of people with AD have hand eczema at some time in their life. Rashes on the feet, scalp or behind the ears are other clues that might point to AD. 4. The appearance of the rash is probably the least helpful clue, because it may be very different from one person to another. Scratch marks are often seen, along with scaly dry skin. The skin may become infected and show yellow crusts or little, pinpoint, pus-containing bumps. The skin also may get very thickened from long-term scratching and rubbing. 5. Heredity. If other family members or relatives have AD, asthma or hay fever, the diagnosis of AD is more likely.

Does it run in families?

AD is a familial disease, though the exact way it passes from parents to children is unclear. If one parent has AD, or any of the other atopic diseases (asthma, hay fever), the chances are about 50% that the child will have one or more of the diseases. If both parents are atopic, chances are even greater that their child will have it. However, the connection is not an absolute one: As many as 30% of affected patients have no family members with any of these allergic disorders. What are the causes of atopic dermatitis?

The exact cause of atopic dermatitis is not known. Caregivers do not know why cells overreact and cause a lot of swelling. Atopic dermatitis is more likely to occur if another family member has eczema, hay fever, asthma, or allergies. The following conditions and substances may trigger a flare- up of your child's atopic dermatitis. Although it is such a common disease, relatively little is understood about the underlying causes of atopic eczema. While AE is associated with allergic asthma and allergic rhinitis, the connection between the diseases has not been established. Twin studies have consistently shown that the disease has a higher rate of concordance in identical as compared to fraternal twins, which also indicates that genetics plays a role in its development. However, the rate of concordance between identical twins is far from 100%, and the changing frequency of the disease over time points to the environmental factors—nutrition or hygiene, for instance—that also play a role in disease susceptibility.

Genomic research into the cause of multigenic diseases is still in its infancy: few genes have ever been identified that contribute to multigenic human disorders. Researchers have attempted to do this in past whole-genome screens for AE and related diseases, but their results have been inconsistent. A few of the pertinent loci have been validated by replication in further studies (chromosome 2q, chromosome 6p, and chromosome 12q, for example), but most have not been.

Associations with ATOD1, ATOD2, ATOD3, ATOD4, ATOD5 and ATOD6 have been identified.

AD is not contagious. People with AD cannot "give" it to someone else.

AD inflammation results from too many reactive inflammatory cells in the skin. Research is seeking the reason why these cells over-react. Patients with AD (or asthma or hay fever) are born with these 4 over-reactive cells. When something triggers them, they don’t turn off as they should. We try to control AD by preventing the trigger factors that turn on inflamed skin, or by "damping the flames" with anti-inflammatory therapies.

What are the trigger factors?

Trigger factors may be different for different people. Most children get worse when they get a cold or other infection. Most have worse problems in the winter; but others simply can’t stand the sweating during hot, humid summer weather. Let’s look at the trigger factors that seem to affect every child with AD.

Dry skin. The skin’s main function is to provide a barrier against dirt, germs and chemicals from the outside. We don’t notice this barrier unless it gets dry, and then it’s scaly, rough and tight. Dry skin is brittle — moist skin is soft and flexible. People with AD have a defect in their skin and it won’t stay moist. It is especially bad in winter when the heat is on in the house and the humidity drops. Other things that dry the skin are too much bathing without proper moisturizing. The challenge: Prevent skin dryness.

Irritants. Irritants are any of the substances outside the body that can cause burning, redness, itching or dryness of the skin. The challenge: Avoid irritating substances.

Stress. Emotional stress comes from many situations. People with AD often react to stress by having red flushing and itching. Special problems for children with AD include frustration, anger or fear, such as when getting the "silent treatment" from a parent. And, of course, AD itself, and its treatments, are a source of stress! The challenge: Recognize stress and reduce it.

Heat and sweating. Most people with atopic dermatitis notice that when they get hot, they itch. They have a type of prickly heat that doesn’t occur just in humid summertime but any time they sweat. It can happen from exercise, from too many warm bedclothes or rapid changes in temperature from cold to warm.

Infections. Bacterial "staph" infections are the most common, especially on arms and legs. Such infections might be suspected if areas are weeping or crusted or if small "pus-bumps" are seen. Herpes infections (such as fever blisters or cold sores) and fungus (ringworm or athlete’s foot) can also trigger AD. If some lesions look different, ask your doctor. If they turn out to be infected, they can be treated with antibiotics. Recognize and treat pustules or crusted lesions.

Allergens. Allergens are materials such as pollen, pet dander, foods, or dust, that cause allergic responses. Allergic diseases such as asthma and hay fever, which flare quickly, are easy to tie to allergens. Itching and hives appear soon after exposure to these airborne allergens and last only briefly. The slower, continuing, chronic eczema of AD may be more difficult to tie to specific allergens. In general, food allergies trigger AD only in children, mainly those with severe disease. Pollens, dust mites and pets can seldom be shown to trigger eczema. Scratch tests are only brief reactions and do not diagnose allergen-triggered eczema. Patch tests can diagnose eczema responses in some cases such as allergies to skin care products.

Are there other trigger factors?

Children with AD will be helped by reducing the major trigger factors described above. But individuals may be subject to other trigger factors, and it is important to be alert for these, too. They may be less common, but can be very damaging for a given child.

How can you avoid trigger factors?

5 1. Keep the skin barrier intact. MOISTURIZE! 2. Wear soft clothes that "breathe." Avoid fabrics of wool, nylon, or stiff material. 3. If sweating causes itch, find ways to keep cooler: o Reduce exertion, especially during times of flare. o Layer clothing and adjust to temperature changes. o Don’t overheat rooms, especially the bedroom. o Use light bedclothes. 4. When itching from sweating, dust, pollen or other exposures, take a cooling shower or tub bath. 5. Learn to recognize signs of infection and treat early. 6. If you suspect food allergy, be systematic. Likely offenders are eggs, milk, peanuts, soy, and seafood, but any food can do it. Can you exclude the most likely offender for a week? Substitute hydrolysate (e.g. Alimentum® or Nutramagen®) for cow milk formula. Keep a food diary. When the skin clears up, try the food. Watch for signs of itching or redness over the next two hours. Do not try a suspect food if it causes hives or face swelling. Don’t exclude multiple food groups at the same time — it’s rare to have more than one or two food allergies, and your child can get malnourished with prolonged avoidance of many foods. 7. With allergy-prone kids, furry animals are a risk. If you must have pets, keep them outside or at least off beds, rugs and furniture where the child plays. Dust mites collect in bedroom carpets and bedding. Simple control measures include coverings for pillows and mattresses, removing bedroom carpets and frequent washing of bedclothes in hot water. 8. Think about stress-causing events and ways to cope with them. Review problems with your doctor or a mental health professional. Try to make AD treatments part of a daily, family routine. Encourage children with AD to do what they can on their own.

A decreased incidence of infections in the first year of life may also contribute to an increased risk for atopy. Children with tuberculosis appear to have a decreased risk of atopy. During tuberculous infection, there is a preferential stimulation of Th1 cells, at the expense of Th2 cell expression. Th2 cells appear to be central to the pathogenesis of atopic disease, and therefore anything that "suppresses" Th2 expression may help inhibit development of asthma, eczema, or allergic rhinitis. It remains to be seen whether less infection in early life will lead to more atopy, but some data are suggestive.

Although the exact pathogenesis of atopic disease is unclear, Th2 cells play an important role. These cells elaborate cytokines, which recruit eosinophils to affected areas. Adhesion molecules may also be important. Their expression appears to be upregulated on endothelial cells of patients with atopic dermatititis. Adhesion molecules facilitate migration of leukocytes from the vascular compartment to surrounding tissues. The level of soluble adhesion factor in the circulation appears to correlate with expression on endothelial cells. A European study evaluating the effect of early treatment on atopic dermatitis in childhood noted that serum levels of E-selectin, a subset of intercellular adhesion molecules (ICAMs), correlated well with disease severity.

Genetic factors clearly play a role in the expression of disease, and a child is at significantly increased risk for atopic conditions if one or both parents are atopics. However, the role of food and animal protein allergies is more controversial. One controlled intervention study by Hide et al from the Isle of Wight eliminated exposure to cow's milk, peanut, and dust mite antigen exposure in 1-year-old children. The investigators documented a decrease in prevalence of both eczema and asthma at 4 years of life in the intervention group compared with the controls. A small subset of patients may benefit from elimination diet and/or environmental modification. Such interventions are not generally recommended as first-line intervention for mild eczema, partially because of the difficulty in carrrying out strict elimination diets, and also because current testing to identify such allergens has a high false- positive rate.

Diagnosis

6 The diagnosis of atopic dermatitis is based on the findings of the history and physical examination. Exposure to possible exacerbating factors, such as aeroallergens, irritating chemicals, foods and emotional stress, should be investigated.

Unfortunately, no specific laboratory findings or histologic features define atopic dermatitis. Although elevated IgE levels are found in up to 80 percent of affected patients, IgE levels are also elevated in patients with other atopic diseases.

Diagnostic Features of Atopic Dermatitis* Major features Pruritus Chronic or relapsing dermatitis Personal or family history of atopic disease Typical distribution and morphology of atopic dermatitis rash: Facial and extensor surfaces in infants and young children Flexure lichenification in older children and adults Minor features Eyes Cataracts (anterior subcapsular) Keratoconus Infraorbital folds affected Facial pallor Palmar hyperlinearity Xerosis Pityriasis alba White dermatographism Ichthyosis Keratosis pilaris Nonspecific dermatitis of the hands and feet Nipple eczema Positive type I hypersensitivity skin tests Propensity for cutaneous infections Elevated serum IgE level Food intolerance Impaired cell-mediated immunity Erythroderma Early age of onset *--The diagnosis of atopic dermatitis should be suspected if three major criteria and three minor criteria are present.

The diagnosis requires the presence of at least three major features and at least three minor features.

Pruritus is a universal finding in atopic dermatitis. The pruritus can be severe, sometimes causing sleep disruption, irritability and generalized stress for affected patients and family members. Pruritus leads to scratching that results in secondary skin changes such as lichenification (thick accentuation of skin lines), excoriation and breakdown of the skin barrier. Consequently, atopic dermatitis has been referred to as "the itch that rashes" rather than the "rash that itches."

The skin lesions observed in atopic dermatitis vary greatly, depending on the severity of inflammation, different stages of healing, chronic scratching and frequent secondary infections. Acute lesions are papules and vesicles on a background of erythema. Subacute lesions may develop scales and lichenification. Chronically involved areas become thick and fibrotic, and nodules often develop 7 within the plaques. Lesions at any stage can develop secondary infections, which may resemble impetigo, with crusting and weeping lesions. When the lesions resolve, areas of hyperpigmentation or hypopigmentation persist.

Xerosis (dry skin) is another characteristic skin finding in patients with atopic dermatitis. Because xerotic skin is unable to hold moisture, it is less pliable and more likely to crack and fissure. Resultant skin barrier breakdown increases susceptibility to irritation and infection. Reversing xerosis is one of the key elements in the treatment of atopic dermatitis.

In infants and young children with atopic dermatitis, pruritus commonly is present on the scalp, face (cheeks and chin) and extensor surfaces of the extremities. Older children and adults typically have involvement of the flexor surfaces (antecubital and popliteal fossa), neck, wrists and ankles. The presence of extensor distribution in older children and adults indicates a poor prognosis for ultimate cure.

Common periocular findings include periorbital hyperpigmentation and Dennie-Morgan folds (prominent folds of skin under the lower eyelid). Anterior subcapsular cataracts develop in 4 to 12 percent of patients with atopic dermatitis. Posterior cataracts are usually a side effect of oral corticosteroid therapy or of topical corticosteroids used in the periorbital area.9

From 50 to 80 percent of patients with atopic dermatitis have or develop asthma or allergic rhinitis. Nearly 70 percent of patients have a positive family history of atopy. Even if atopic dermatitis resolves with age, the predisposition for asthma and rhinitis persists.

Differential Diagnosis

Because the skin lesions in atopic dermatitis can take many forms (papules, vesicles, plaques, nodules and excoriations), the differential diagnosis of atopic dermatitis is extensive. Conditions that need to be considered in patients with pruritus include seborrheic dermatitis, and neurodermatitis. Features that distinguish these and other conditions from atopic dermatitis are listed in.

Systemic illnesses such as malignancy, thyroid disorders and hepatic or renal failure can also cause pruritus and excoriations. In adults with new-onset pruritus, a thorough history and a complete physical examination are necessary to exclude systemic disease.

Occasionally, a patient with skin lesions and a history consistent with atopic dermatitis will have contact dermatitis. If the history reveals exacerbation of the rash after contact with a particular substance or if standard therapy is unsuccessful, patch testing to identify a cause of allergic contact dermatitis may be indicated. Differential Diagnosis of Atopic Dermatitis

Disease Distinguising features Seborrheic dermatitis Greasy, scaly lesions, absence of family history Psoriasis Localized patches on extensor surfaces, scalp, buttocks; pitted nails Neurodermatitis Usually, a single patch in an area accessible to itching; absence of family history Contact dermatitis Positive exposure history, rash in area of exposure, absence of family history Scabies Papules, finger web involvement, positive skin scraping Systemic Findings on complete history and physical examination vary by disease Dermatitis herpetiformis Vesicles over extensor areas and associated enteropathy

8 Dermatophyte infection Serpiginous plaques with central clearing, positive potassium hydroxide preparation Immunodeficiency disorder History of recurrent infection

SCORAD ("SCORing Atopic Dermatitis") is a clinical tool for assessing the severity (i.e. extent, intensity) of atopic dermatitis as objectively as possible. It was developed by the European Task Force on Atopic Dermatitis in 1993.

One difficulty in assessing any form of intervention in atopics is the difficulty in accurately quantitating degree and extent of disease in individuals. A reliable scoring system (widely accepted in Europe) has been developed to better quantitate disease. This system, referred to as SCORAD, may enable centers to better define response to therapy and more easily compare data from different centers. However, this system is relatively time-consuming, and a simpler method, the 3-item severity score (TIS), has been advocated as a reasonable alternative for routine use. This system uses degree of erythema, edema, and excoriations to categorize the extent and severity of patient disease as either mild, moderate, or severe. Treatment

The treatment of atopic dermatitis targets underlying skin abnormalities such as xerosis, pruritus, superinfection and inflammation. Patients should also be educated about the chronic nature of the disease and the need for continued adherence to proper skin care. Most patients with atopic dermatitis respond to a regimen that uses emollients and topical steroids. Antihistamines and antibiotics are also useful adjuncts. More severe cases may require allergen avoidance and more intense education and psychological support for the family. A "fourth generation" of topical steroids, including both mometasone and fluticasone, appear to have an improved therapeutic index with less adverse effects noted after use for more than 2 weeks. Nonetheless, adverse effects, including striae and hypertrichosis at the site of application, as well as adrenal axis suppression, can occur with prolonged or inappropriate use of these drugs. Fluticasone cream .05% diluted with varying proportions of emollient has proven particularly effective when used under "wet wraps." This form of treatment has been used in children with severe eczema who have not responded to routine topical treatment. The affected child is bathed, then covered with the diluted fluticasone/emollient combination. Wet gauze or cloth is then applied in strips over the affected areas, and finally the affected sites are covered with either dry gauze or clothing such as pajamas. The sites may be rehydrated as frequently as every 2 hours, but can also be left in place untouched overnight. Eighteen children treated in this fashion showed marked improvement after just 1 week of treatment. Three of the 18 showed mild suppression of fasting cortisol levels. Such treatment can be continued in day care settings and taught to parents. Other therapies that have been used in severe disease include phototherapy and cyclosporine. Cyclosporin is typically used for 3 months and patients must be carefully followed for renal toxicity. Rare cases of markedly increased alkaline phosphatase have also been reported. Relapse usually occurs shortly after discontinuation of therapy. Cetirizine, a new oral antihistamine that is a metabolite of hydroxizine, has proven useful in decreasing the incidence of urticaria and the need for more potent topical steroids in atopics. This drug has a prolonged half-life, can be taken once a day, and in high doses inhibits eosinophil chemotactic factor. In a study evaluating 807 children with dust mite and grass pollen allergies, use of this drug decreased the incidence of development of asthma in the treated group by 50%. Topical agents that hold promise include tacrolimus and ascomycin. Both of these immunomodulatory drugs have demonstrated efficacy when used in the treatment of moderate and severe atopic dermatitis. Thus far, toxicity has been minimal with the use of either agent. Atopic dermatitis continues to be a therapeutic challenge, but a better understanding of its pathogenesis and more efficacious therapies have markedly improved the clinician's ability to treat this common disease.

9 Treatment of atopic dermatitis.

Corticosteroids Systemic corticosteroids should be reserved for use in patients with severe treatment-resistant atopic dermatitis. Oral corticosteroids improve the lesions of atopic dermatitis, but a disease flare may occur when these are stopped. If a systemic corticosteroid is used to treat a severe flare of atopic dermatitis, the potential for a rebound effect can be decreased by tapering the drug while increasing topical corticosteroid treatment and aggressively hydrating the skin. Topical corticosteroids are effective in patients with atopic dermatitis, but therapy with these agents should not replace the frequent use of moisturizers. The local and systemic side effects of topical steroids are well recognized. Local effects include skin atrophy, striae, telangiectasias, 10 hypopigmentation, rosacea, perioral dermatitis and acne. Systemic side effects include adrenal suppression, cataracts, glaucoma and growth retardation in children. The risk of side effects from corticosteroids depends on multiple factors, including the potency of the steroid, the vehicle, the amount of steroid used, the concomitant use of occlusion, the area being covered and the integrity of the skin. The greatest penetration occurs with steroid use on the groin and face; the lowest penetration occurs with application on the palms and soles. Topical corticosteroids are grouped into seven potency categories, with group 1 containing the most potent agents and group 7 containing the least potent agents. A general principle in treating atopic dermatitis with topical steroids is to use the least potent agent possible and limit the frequency of application. Compared with adults, children (especially infants) are at higher risk for the local and systemic side effects of topical corticosteroids. Thus, it is reasonable to use a group 6 or 7 steroid initially in infants and for intertriginous areas in patients of any age. If the dermatitis is severe and a more potent steroid is needed, the patient should be followed closely, and the strength of the steroid should be reduced as skin lesions improve. Use of a mid-potency topical corticosteroid (group 4 or 5) is appropriate for nonintertriginous areas in children and adults. Group 1 and 2 steroids are best reserved for use on thickened plaques and for the palms and soles. Group 1 steroids are generally best avoided in children under 12 years of age or for use with occlusion. Topical steroids come in many vehicles, including solutions, lotions, creams, gels and ointments. The thicker preparations (ointments) penetrate the epidermis better. The same steroid in a different vehicle can differ in potency by one or two classes. Some areas of the body dictate the type of vehicle needed. For example, the scalp is best treated with solutions and lotions. Occlusion should be limited to isolated resistant areas because it significantly increases the absorption of topical steroids.

Tar Preparations Tar preparations have anti-inflammatory and effects on the lesions of atopic dermatitis. These preparations are effective when used alone or with topical corticosteroids. Some tar preparations, especially gels, may contain alcohol and thus can be irritating. Shampoos, bath solutions and creams are less irritating. Tar preparations can be purchased over the counter (e.g., Cutar, Aquatar, Estar). The disadvantages of tars are their odor and dark staining color. Using the products at night and covering the treated areas can decrease these problems.

BIBLIOGRAPHY

BASIC 1. Behrman Richard E., Robert M. Kliegman, Hal B. Jenson, eds. Nelson Texbook of Paediatrics. Philadelfia: WB Saunders, 18th edition - 2014. – 994 р. – Р.638. 2. Buryak V.N., Makhmutov R.F., Poshehonova J.V. Children`s diseases. – Donetsk: Nord-Press, 2010. – 328 p. 3. Pediatry. Guidance aid / Ed. by O. Tiazhka – Kyiv: Medicine, 2007. 4. Рediatrics: textbook/ T.O. Kryuchko, O.Y. Abaturov, T.V. Kushnereva, at all. Edited by T.O. Kryuchko, O.Y. Abaturov. Kyiv: AUS Medicine Publishing, 2016. - 208 p.+ 2p. of color insert.

ADDITION 1. Illustrated Textbook of Paediatrics/ Ed.: T. Lissauer, G. Clayden.- Edinburg: Mosby, 2006.- 410 pp. 2. Forfar & Arneil’s Textbook of Pediatrics/ Ed.: N.Mc Intosh, P.J. Helms, R.L. Smyth, S. Logan.- Edinburg: Churchill Livingstone, 2014. - 1687 pp.

THEMATIC SITES: http://www.freebookcentre.net/medical_text_books_journals/pediatrics_online_texts_download.html http://pediatrics.aappublications.org 11 http://www.generalpediatrics.com http://www.aap.org/en-us/Pages/Default.aspx http://hospitalpediatrics.org

Methodical instructions were discussed and approved at the meeting of department of Pediatrics №2 protocol № ___ of ______20____.

Additions and changes were made “____” ______20____ Additions and changes were made “____” ______20____ Additions and changes were made “____” ______20____ Additions and changes were made “____” ______20____

The methodical instructions are compiled by Associate Prof Poda O.A.

Methodical instructions were discussed and approved at the meeting of department of Pediatrics №2 protocol № ___ of ______20____. Additions and changes were made “____” ______20____ Additions and changes were made “____” ______20____ Additions and changes were made “____” ______20____ Additions and changes were made “____” ______20____ Additions and changes were made “____” ______20____

12 MINISTRY OF HEALTH OF UKRAINE UKRAINIAN MEDICAL STOMATOLOGICAL ACADEMY

“APPROVED” at the meeting of department of Pediatric №2 “ ______05__” ____06 2020 Protocol № 21 Head of the department MD, Prof. Kryuchko T.O. ______

Methodical instructions for independent work of students during preparation to practical classes and in class

Academic discipline Pediatrics

Module # 3 Topic of the lesson Asthma in children Course 4 Faculty Medical

Poltava 2020 1. Actuality of the subject. Asthma is one of the most common chronic diseases globally and currently affects ~300 million people. The prevalence of asthma has risen in affluent countries over the last 30 years but now appears to have stabilized, with ~10–12% of adults and 15% of children affected by the disease. In developing countries where the prevalence of asthma had been much lower, there is a rising incidence that appears to be associated with increased urbanization. The prevalence of atopy and other allergic diseases has also increased over the same time, suggesting that the reasons for the increase are likely to be systemic rather than confined to the lungs. This epidemiologic observation suggests that there is a maximum number of individuals in the community who are liable to be affected by asthma, likely by genetic predisposition. Most patients with asthma in affluent countries are atopic, with allergic sensitization to the house dust mite. Asthma affects about 5 percent of the population younger than 18 years. Fifty to 80 percent of children with asthma develop symptoms before five years of age.

2. Concrete aims: 2.1 Define the patient's complaints. 2.2 Collect anamnesis (history) disease. 2.3. Be able to conduct the chest palpation, percussion and auscultation of lungs. 2.4. Know classification of asthma. 2.5. Identify the characteristic signs of asthma in different age of a child. 2.6. Make up a plan of investigation of a child with asthma. 2.7. Be able to estimate general and biochemical blood analysis, nose and throat secretion investigation, pleural fluid investigation, to interpret functional lung investigations (spirography, oxygenometria), X-ray investigation. 2.8. Make a preliminary diagnosis of asthma. 2.9. Give basic, pathogenetic and symptomatic therapy asthma. 2.10. To demonstrate the usage by medical specialist of moral-deontological principles in child's pulmonology.

3. Basic knowledge, skills, habits necessary for studying the subject (interdisciplinary integration).

Names of previous disciplines Acquired skills and habits 1. Anatomy, histology Аnatomic and histological features of respiratory system at children 2. Physiology Peculiarities of the respiratory system and gaseous exchange in child organism in norm and pathology. 3. Biochemistry To give a clinical estimation of the changes of biochemical indexes of blood. 4. Propadeutic pediatrics Characteristic complaints, clinical syndromes at the diseases of the respiratory system. Ability to collect anamnesis, find out the special complaints, conduct the clinical examination of children with the diseases of the respiratory system. 5. Pharmacology To have knowledge about the basic groups of medical preparations which are used at treatment of asthma .

6. Public Health To use knowledge about the structure of medical help for child's population for the proper use of resources of the system of health protection in the plan of prophylaxis and treatment.

4. Tasks for independent work during preparation for the study: 4.1. The enumeration of the main terms, parameters, characteristics which the student is to assimilate while preparing for the class:

Term Definition 1. Cyanosis Rate of oxygen deficiency in the blood 2. Respiratory rate (bpm) Newborn - 40 - 60, 1 year 30 - 35, 5 years - 25, 10 years - 20, over 12 years - 20 – 16 3. Dyspnea Shortness of breath with a violation of its frequency, depth and rhythm 4. Types of breathlessness inspirator, expiratory, mixed 5. Types rales Moist, wheezing 6. The degrees of respiratory I, II, III insufficiency 7. Spirography Method of graphic registration breath movements, indicating changes in lung volume 8. Types of pleurisy Dry and exudative 9. Artificial Respiration breathing mouth to mouth, and mouth to nose 10. Tachypnea Increased respiratory rate more than 10% 11. Peak expiratory flow rate Way to diagnose the degree of airway obstruction asthma. Subjects (PEFR) are asked to take a full inspiration to total lung capacity and then blow out forcefully into the peak flow meter.

4.2. Theoretical questions for the class: 1. What is asthma? What is GINA? 2. What factors influence on the development and expression of asthma? 3. Tell me more in details about host factors?  What are the main pathogenetic areas which can be inherited?  Who suffer from asthma more often girls or boys? And why? 4. What environmental factors influence on the development and expression of asthma?  What viruses have been associated with the inception of the asthmatic phenotype?  How does smoking affect the increase of asthma? 5. What are the mechanisms of asthma?  What cells take part in allergy inflammation? 6. What are the symptoms of asthma?  What questions will you ask the patient about the asthma? 7. What is it the cough-variant of asthma? 8. What will you see during the physical examination of asthma? 9. What methods are used for measurements of lung function? 10. What methods are used for measurements of allergic status? 11. How does asthma express in children 5 years and younger? 12. What is the classification of asthma? 13. Let’s speak about the asthma treatment. What the routes of administration are the most popular in children?  Tell about the preferred and alternate devices for children of different age group? 14. What are the controller medications and what treatment is used?  What are the inhaled glucocorticosteroids do you know? How to prescribe them? 15. What are there adverse effects of inhaled glucocorticosteroids do you know? 16. What are there leukotriene modifiers and long-acting inhaled β2-agonists do you know? 17. Why aren’t cromones used? 18. How do you understand reliever medications? What medications are used in this case? 19. When is allergenspecific used? 20. How do you understand “asthma is under control”?

3 4.3. Practical tasks to be carried out in class:

1. Working with test tasks. 2. Work of students in chambers near the bed of sick children with asthma. 3. Interpretation of additional methods of examination. 4. Clinical analysis of model case. 5. Deciding of situation tasks. 6. Setting of treatment for every child with asthma.

5. Content of the topic:

Asthma is a chronic inflammatory condition of the lung airways resulting in episodic airflow obstruction. This chronic inflammation heightens the “twitchiness” of the airways—airways hyperresponsiveness (AHR)—to provocative exposures. Asthma management is aimed at reducing airways inflammation by minimizing proinflammatory environmental exposures, using daily “controller” anti-inflammatory medications, and controlling co-morbid conditions that can worsen asthma. Less inflammation typically leads to better asthma control, with fewer exacerbations and decreased need for “quick-reliever” asthma medications. Exacerbations can, nevertheless, still occur. Early intervention with systemic corticosteroids greatly reduces the severity of such episodes. Advances in asthma management and especially pharmacotherapy enable all but the uncommon child with severe asthma to live normally.

ETIOLOGY. Although the cause of childhood asthma has not been determined, contemporary research implicates a combination of environmental exposures and inherent biological and genetic vulnerabilities. Respiratory exposures in this causal environment include inhaled allergens, respiratory viral infections, and chemical and biological air pollutants such as environmental tobacco smoke. In the predisposed host, immune responses to these common exposures can be a stimulus for prolonged, pathogenic inflammation and aberrant repair of injured airways tissues. Lung dysfunction (i.e., AHR and reduced airflow) develops. These pathogenic processes in the growing lung during early life adversely affect airways growth and differentiation, leading to altered airways at mature ages. Once asthma has developed, ongoing exposures appear to worsen it, driving disease persistence and increasing the risk of severe exacerbations.

Early Childhood Risk Factors for Persistent Asthma:  Parental asthma Allergy  Pneumonia  Atopic dermatitis  Bronchiolitis requiring hospitalization  Allergic rhinitis  Wheezing apart from colds  Food allergy  Male gender  Inhalant allergen sensitization  Low birthweight  Food allergen sensitization  Environmental tobacco smoke exposure  Severe lower respiratory tract infection  Reduced lung function at birth.

PATHOGENESIS. Airflow obstruction in asthma is the result of numerous pathologic processes. In the small airways, airflow is regulated by smooth muscle encircling the airways lumens; bronchoconstriction of these bronchiolar muscular bands restricts or blocks airflow. A cellular inflammatory infiltrate and exudates distinguished by eosinophils, but also including other inflammatory cell types (neutrophils, monocytes, lymphocytes, mast cells, basophils), can fill and obstruct the airways and induce epithelial damage and desquamation into the airways lumen. Helper T lymphocytes and other immune cells that produce pro-allergic, proinflammatory cytokines (IL-4, IL-5, IL-13) and chemokines (eotaxin) mediate this inflammatory process. Pathogenic immune responses and inflammation may also result from a breach in normal immune regulatory processes (regulatory T lymphocytes that produce IL-10 and transforming growth factor [TGF]–β) that dampen effector immunity and inflammation when they are no longer needed. Airways inflammation is linked to AHR or hypersensitivity of airways smooth muscle to numerous provocative exposures that act as triggers, 4 as well as airways edema, basement membrane thickening, subepithelial collagen deposition, smooth muscle and mucous gland hypertrophy, and mucus hypersecretion—all processes that contribute to airflow obstruction.

CLINICAL MANIFESTATIONS AND DIAGNOSIS. Intermittent dry coughing and/or expiratory wheezing are the most common chronic symptoms of asthma. Older children and adults will report associated shortness of breath and chest tightness; younger children are more likely to report intermittent, nonfocal chest “pain.” Respiratory symptoms can be worse at night, especially during prolonged exacerbations triggered by respiratory infections or inhalant allergens. Daytime symptoms, often linked with physical activities or play, are reported with greatest frequency in children. Other asthma symptoms in children can be subtle and nonspecific, including self-imposed limitation of physical activities, general fatigue (possibly due to sleep disturbance), and difficulty keeping up with peers in physical activities. Asking about previous experience with asthma medications (bronchodilators) may provide a history of symptomatic improvement with treatment that supports the diagnosis of asthma. Lack of improvement with bronchodilator and corticosteroid therapy is inconsistent with underlying asthma and should prompt more vigorous consideration of asthma-masquerading conditions. During asthma exacerbations, expiratory wheezing and a prolonged expiratory phase can usually be appreciated by auscultation. Decreased breath sounds in some of the lung fields, commonly the right lower posterior lobe, are consistent with regional owing to airways obstruction. Crackles (or rales) and rhonchi can sometimes be heard, resulting from excess mucus production and inflammatory exudate in the airways. The combination of segmental crackles and poor breath sounds can indicate lung segmental atelectasis that is difficult to distinguish from bronchial pneumonia and can complicate acute asthma management. In severe exacerbations, the greater extent of airways obstruction causes labored breathing and respiratory distress manifested as inspiratory and expiratory wheezing, increased prolongation of exhalation, poor air entry, suprasternal and intercostal retractions, nasal flaring, and accessory respiratory muscle use. In extremis, airflow may be so limited that wheezing cannot be heard. DIFFERENTIAL DIAGNOSIS. Many childhood respiratory conditions can present with symptoms and signs similar to asthma. Besides asthma, other common causes of chronic, intermittent coughing include rhinosinusitis and gastro-esophageal reflux (GER). Both GER and chronic sinusitis can be challenging to diagnose in children. Often, GER is clinically silent in children, and children with chronic sinusitis do not report sinusitis-specific symptoms such as localized sinus pressure or tenderness. In addition, both GER and rhinosinusitis are often co-morbid conditions with childhood asthma and, if not specifically treated, make asthma difficult to manage.

Differential Diagnosis of Childhood Asthma UPPER RESPIRATORY TRACT CONDITIONS Allergic rhinitis Chronic rhinitis Sinusitis Adenoidal or tonsillar hypertrophy Nasal foreign body MIDDLE RESPIRATORY TRACT CONDITIONS Laryngotracheobronchomalacia Laryngotracheobronchitis (e.g., pertussis) Laryngeal web, cyst, or stenosis Vocal cord dysfunction

5 Vocal cord paralysis Tracheoesophageal fistula Vascular ring, sling, or external mass compressing on the airway (e.g., tumor) Foreign body aspiration Chronic bronchitis from environmental tobacco smoke exposure Toxic inhalations LOWER RESPIRATORY TRACT CONDITIONS Bronchopulmonary dysplasia (chronic lung disease of preterm infants) Viral bronchiolitis Gastroesophageal reflux Causes of bronchiectasis: Cystic fibrosis Immune deficiency Allergic bronchopulmonary mycoses (e.g., aspergillosis) Chronic aspiration Immotile cilia syndrome, primary ciliary dyskinesia Bronchiolitis obliterans Interstitial lung diseases Hypersensitivity pneumonitis Pulmonary eosinophilia, Churg-Strauss vasculitis Pulmonary hemosiderosis Tuberculosis Pneumonia Pulmonary edema (e.g., congestive heart failure) Medications associated with chronic cough Acetylcholinesterase inhibitors β-adrenergic antagonists

In early life, chronic coughing and wheezing can indicate recurrent aspiration, tracheobronchomalacia, a congenital anatomic abnormality of the airways, foreign body aspiration, cystic fibrosis, or bronchopulmonary dysplasia. In older children and adolescents, vocal cord dysfunction (VCD) can present as intermittent daytime wheezing. In this condition, the vocal cords close inappropriately, during inspiration and sometimes exhalation, producing shortness of breath, coughing, throat tightness, and often audible laryngeal wheezing and/or stridor. In most VCD cases, spirometric lung function testing will reveal “truncated” and inconsistent inspiratory and expiratory flow-volume loops, a pattern that differs from the reproducible pattern of airflow limitation in asthma that improves with bronchodilators. VCD may also be visualized with laryngoscopy. VCD can coexist with asthma. VCD does not respond to traditional asthma therapy. Speech therapy is the treatment of choice for VCD.

LABORATORY FINDINGS.

6 Lung function tests can help to confirm the diagnosis of asthma and determine disease severity. Pulmonary Function Testing. Forced expiratory airflow measures are helpful in diagnosing and monitoring asthma and in assessing efficacy of therapy. Lung function testing is particularly helpful in children with asthma who are poor perceivers of airflow obstruction or when physical signs of asthma do not occur until airflow obstruction is severe. Many asthma guidelines promote spirometric measures of airflow and lung volumes during forced expiratory maneuvers as standard for asthma assessment. Spirometry is helpful as an objective measure of airflow limitation. Knowledgeable personnel are needed to perform and interpret spirometry tests. Valid spirometric measures are dependent on a patient's ability to perform properly a full, forceful, and prolonged expiratory maneuver, usually feasible in children >6 yr of age (with some younger exceptions). Reproducible spirometric efforts are an indicator of test validity; if, on 3 attempts, the FEV1 (forced expiratory volume in 1 sec) is within 5%, then the highest FEV1 effort of the 3 is used. This standard utilization of the highest of 3 reproducible efforts is indicative of the effort-dependence of reliable spirometric testing. In asthma, airways blockage results in reduced airflow with forced exhalation and smaller partial- expiratory lung volumes. Because asthmatics are typically hyperinflated, FEV1 can be simply adjusted for full expiratory lung volume—the forced vital capacity (FVC)—with an FEV1/FVC ratio. Generally, an FEV1/FVC ratio <0.80 indicates significant airflow obstruction. Normative values for FEV1 have been determined for children, based on height, gender, and ethnicity. Abnormally low FEV1 as a percentage of predicted norms is 1 of 4 criteria used to determine asthma severity in the National Institutes of Health (NIH)–sponsored asthma guidelines. The guidelines cutoff criteria of FEV1 <80% and <60% of predicted for moderate and severe asthma, respectively, are controversial for children with asthma, many of whom can have near-normal or even supra-normal airflow despite having the other hallmarks of moderate to severe disease.

Measuring exhaled nitric oxide (FENO), a marker of airway inflammation in asthma, can help titrate medications and confirm the diagnosis of asthma.

Peak expiratory flow (PEF) monitoring devices provide a simple and inexpensive home-use tool to measure airflow and can be helpful in a number of circumstances. “Poor perceivers” of airflow obstruction due to asthma can benefit by monitoring PEFs daily to assess objectively airflow as an indicator of asthma control or problems that would be more sensitive than their symptom perception. PEFs vary in their ability to detect airflow obstruction; in some patients, PEFs decline only when airflow obstruction is severe. Therefore, PEF monitoring should be started by measuring morning and evening PEFs (best of 3 attempts) for several weeks for patients to practice the technique, to determine a “personal best,” and to correlate PEF values with symptoms (and ideally spirometry). PEF variation >20% is consistent with asthma.

Radiology. Chest radiographs (posteroanterior and lateral views) in children with asthma often appear to be normal, aside from subtle and nonspecific findings of hyperinflation (flattening of the diaphragms) and peribronchial thickening. Chest radiographs can be helpful in identifying abnormalities that are hallmarks of asthma masqueraders (aspiration pneumonitis, hyperlucent lung fields in bronchiolitis obliterans), and complications during asthma exacerbations (atelectasis, pneumomediastinum, pneumothorax). Some lung abnormalities can be better appreciated with high- resolution, thin-section chest CT scans. Bronchiectasis is sometimes difficult to appreciate on chest radiograph, but is clearly seen on CT scan and implicates an asthma masquerader such as cystic fibrosis, allergic bronchopulmonary mycoses (aspergillosis), ciliary dyskinesias, or immune deficiencies.

Other tests, such as allergy testing to assess sensitization to inhalant allergens, help with the management and prognosis of asthma. In a comprehensive U.S. study of 5–12 yr old asthmatic children (Childhood Asthma Management Program [CAMP]), 88% had inhalant allergen sensitization by allergy prick skin testing. 7 TREATMENT. The key elements to optimal asthma management are well recognized. The NIH National Heart, Lung and Blood Institute (NHLBI) developed current asthma management guidelines. For childhood asthma, a publication of the American Academy of Allergy, Asthma and Immunology, the American Academy of Pediatrics, and the NIH, entitled Pediatric Asthma: Promoting Best Practice, has been updated to reflect the 2002 National Asthma Education and Prevention Program (NAEPP) Update. These guidelines describe 4 principle components to optimal asthma management

Principles of Asthma Pharmacotherapy. The NAEPP guidelines offer a stepwise approach to management based on asthma severity categorized as mild intermittent, mild persistent, moderate persistent, and severe persistent asthma. The classification of asthma severity is based on the following parameters: (1) frequency of daytime and (2) nighttime symptoms, (3) degree of airflow obstruction by spirometry, and/or (4) PEF variability. For younger children (<5 yr of age), management is primarily based on symptoms since young children cannot perform the maneuvers required for conventional lung function measurements. A major objective of this approach is to identify and treat all “persistent” asthma with anti-inflammatory controller medication. The type(s) and amount(s) of daily controller medications to be used are determined by the asthma severity rating. The “three strikes” rule is a handy memory aid for determining if an asthmatic child should receive controller therapy. Simply put, if an asthmatic child has asthma symptoms or uses quick-relief medication at least 3 times per wk, awakens at night due to asthma at least 3 times per mo, requires a refill for a quick-relief inhaler prescription at least 3 times per yr, experiences asthma exacerbations at least 3 times per yr, or requires short courses of systemic corticosteroids at least 3 times a yr, then that patient should receive daily controller therapy. In addition, according to the NAEPP guidelines, controller therapy can be considered for children who present with frequent exacerbations (at least 2 exacerbations occurring <6 wk apart). Inhaled corticosteroid (ICS) therapy is recommended as preferred therapy for all levels of asthma severity except for the mild intermittent category. Leukotriene pathway modifiers or sustained- release theophylline (only for patients >5 yr of age) are considered alternative controllers for mild persistent asthmatics. Combination therapy of a low-to-medium dose ICS with a long-acting β-agonist (LABA; preferred) or a leukotriene modifier or theophylline is a mainstay therapy for moderate persistent asthma in older children and adults. While the use of medium-dose ICS alone is an alternative therapy for older children and adults with moderate persistent severity, for infants and young children, it is considered a preferred treatment for moderate persistent asthma. Severe persistent asthmatics should receive high-dose ICS, a long-acting bronchodilator, and routine oral corticosteroids if needed. Daily controller therapy is not recommended for mild intermittent asthma. SABAs are the recommended quick-reliever medications for symptoms and exercise pretreatment for all asthma severity levels. INHALED CORTICOSTEROIDS (ICS). The NAEPP guidelines recommend daily ICS therapy as the treatment of choice for all patients with persistent asthma (see Table 143-9 ). ICS therapy has been shown to reduce asthma symptoms, improve lung function, reduce AHR, reduce “rescue” medication use and, most important, reduce urgent care visits, hospitalizations, and prednisone use for asthma exacerbations by about 50%. ICS therapy may lower the risk of death due to asthma. It can achieve all of the goals of asthma management and, as a result, is viewed as first-line treatment for persistent asthma. There are currently 5 ICSs that are approved by the FDA, and the NAEPP guidelines provide an equivalence classification, although direct comparisons of efficacy and safety outcomes in children are lacking. Newer forms are being developed (mometasone furoate, ciclesonide) that may enhance the efficacy-to-safety profile of ICS therapy while allowing for less frequent dosing. ICSs are available in MDIs, DPIs, or in suspension for nebulization. Fluticasone propionate, mometasone furoate and, to a lesser extent, budesonide are considered “2nd-generation” ICSs in that they have increased anti- inflammatory potency and reduced systemic bioavailability for potential adverse effects, owing to extensive first-pass hepatic metabolism. The selection of the initial ICS dose is based on the determination of disease severity. A fraction of the initial ICS dose is often sufficient to maintain good control after this has been achieved.

8 Although ICS therapy has been widely used in adults with persistent asthma, its application in children has lagged due to concerns of the potential for adverse effects with chronic use. Generally, clinically significant adverse effects that occur with chronic systemic corticosteroid therapy have not been seen or have been only very rarely reported in children receiving ICSs in recommended doses. The risk of adverse effects from ICS therapy is related to the dose and frequency with which ICSs are given. High doses (≥1,000 μg/day in children) and frequent administration (4 times/day) are more likely to cause local and systemic adverse effects. Children who are maintained on higher ICS doses are also likely to require systemic corticosteroid courses for asthma exacerbations, further increasing the risk of corticosteroid adverse effects. SYSTEMIC CORTICOSTEROIDS. ICS therapy has allowed the large majority of children with asthma to maintain good disease control without maintenance (qod) oral corticosteroids. Oral corticosteroid therapy is used primarily to treat asthma exacerbations and in rare patients with severe disease who remain symptomatic despite optimal use of other asthma medications. In these severe asthmatics, every attempt should be made to exclude any co-morbid conditions and to keep the oral corticosteroid dose at ≤20 mg qod. Doses exceeding this amount are associated with numerous adverse effects. To determine the need for continued oral corticosteroid therapy, a taper of the oral corticosteroid dose (over weeks to several months) should be considered, with close monitoring of the patient's symptoms and lung function. When administered orally, prednisone, prednisolone, and methylprednisolone are rapidly and nearly completely absorbed, with peak plasma concentrations occurring within 1–2 hr. Prednisone is an inactive pro-drug that requires biotransformation via first-pass hepatic metabolism to prednisolone, its active form. Corticosteroids are metabolized in the liver into inactive compounds, with the rate of metabolism influenced by drug interactions and disease states. (phenytoin, phenobarbital, carbamazepine) increase the metabolism of prednisolone, methylprednisolone, and dexamethasone, with methylprednisolone most significantly affected. Rifampin also enhances the clearance of corticosteroids and can result in diminished therapeutic effect. Other medications (ketoconazole, oral contraceptives) can significantly delay corticosteroid metabolism. Macrolide antibiotics (erythromycin, clarithromycin, troleandomycin) delay the clearance of only methylprednisolone. Children who require chronically administered oral cortico-steroids are at risk of developing associated adverse effects over time. Essentially all major organ systems can be adversely affected by chronically administered oral corticosteroid therapy. Some of these effects occur immediately (metabolic effects). Others can develop insidiously over several months to years (growth suppression, osteoporosis, cataracts). Most adverse effects occur in a cumulative dose- and duration-dependent manner. Children who require routine or frequent short courses of oral corticosteroids, especially with concurrent high- dose ICSs, should receive corticosteroid adverse effects screening and osteoporosis preventive measures. LONG-ACTING INHALED β-AGONIST (LABA). Although LABAs (salmeterol, formoterol) are β-agonists, they are consid ered to be daily controller medications, not intended for use as “rescue” medication for acute asthma symptoms or exacerbations, nor as monotherapy for persistent asthma. Salmeterol has a prolonged onset of action, with maximal bronchodilation about 1 hr after administration, whereas formoterol has an onset of action within 5–10 min. Both medications have a prolonged duration of effect of at least 12 hr. Given their long duration of action, they are well suited for patients with nocturnal asthma and for individuals who require frequent SABA use during the day to prevent exercise-induced bronchospasm. Their major role is as an “add-on” agent in patients who are suboptimally controlled on ICS therapy alone. For those patients, several studies have found the addition of LABA to ICS to be superior to doubling the dose of ICS, especially on day and nocturnal symptoms. There are also controller formulations that combine ICS with LABA (fluticasone/salmeterol, budesonide/formoterol). LEUKOTRIENE-MODIFYING AGENTS. Leukotrienes are potent pro-inflammatory mediators that can induce bronchospasm, mucus secretion, and airways edema. Two classes of leukotriene modifiers have been developed: inhibitors of leukotriene synthesis and leukotriene receptor antagonists (LTRA). Zileuton, the only leukotriene synthesis inhibitor, is not approved for use in children <12 yr of age. Because zileuton requires administration 4 times daily, can result in elevated 9 liver function enzymes in 2–4% of patients, and interacts with medications metabolized via the cytochrome-P450 system, it is rarely prescribed for children with asthma. LTRAs have bronchodilator and targeted anti-inflammatory properties and reduce exercise-, aspirin-, and allergen-induced bronchoconstriction. They are recommended as an alternative treatment for mild persistent asthma and as an “add-on” medication to ICS for moderate persistent asthma. Two LTRAs are FDA-approved for use in children: montelukast and zafirlukast. Both medications improve asthma symptoms, decrease need for rescue β-agonist use, and improve lung function. Montelukast, which is FDA-approved for use in children ≥1 yr of age, is administered once daily. Zafirlukast is FDA-approved for use in children ≥5 yr of age and is administered twice daily. Although incompletely studied in children with asthma, LTRAs appear to be less effective than ICSs in patients with moderate persistent asthma. In general, ICS improves lung function by 5–15%, whereas LTRA improves lung function by 2–7.5%. LTRAs are not thought to have significant adverse effects, although case reports described a Churg-Strauss–like vasculitis (pulmonary infiltrates, eosinophilia, cardiomyopathy) in adults with cortico-steroid-dependent asthma treated with LTRAs. It remains to be determined whether these patients have a primary eosinophilic vasculitis masquerading as asthma, which was “unmasked” as their oral corticosteroid dose was tapered, or whether the disease is a very rare adverse effect of LTRA. NONSTEROIDAL ANTI-INFLAMMATORY AGENTS. Cromolyn and nedocromil are non- corticosteroid anti-inflammatory agents that can inhibit allergen-induced asthmatic responses and reduce exercise-induced bronchospasm. According to the NAEPP guidelines, both drugs are considered alternative anti-inflammatory drugs for children with mild persistent asthma. Although largely devoid of adverse effects, these medications must be administered frequently (2–4 times/day) and are not nearly as effective daily controller medications as ICSs and leukotriene-modifying agents. Because they inhibit exercise-induced bronchospasm, they can be used in place of SABAs, especially in children who develop unwanted adverse effects with β-agonist therapy (tremor and elevated heart rate). They can also be used in addition to a SABA as a combination pretreatment for exercise-induced bronchospasm in patients who continue to experience symptoms despite SABA pretreatment alone. THEOPHYLLINE. In addition to its bronchodilator effects, theophylline has anti-inflammatory properties as a phosphodiesterase inhibitor, although the extent of their clinical relevance has not been clearly established. Theophylline, when used chronically, can reduce asthma symptoms and the need for rescue SABA use. Although it is considered an alternative monotherapy controller agent for older children and adults with mild persistent asthma, it is no longer considered a first-line agent for small children in whom there is significant variability in the absorption and metabolism of different theophylline preparations, necessitating frequent dose monitoring (blood levels) and adjustments. Because theophylline may have some corticosteroid-sparing effects in individuals with oral corticosteroid-dependent asthma, it is still sometimes used in this group of asthmatic children. Theophylline has a narrow therapeutic window; therefore, when used, serum theophylline levels need to be routinely monitored especially if the patient has a viral illness associated with a fever or is placed on a medication known to delay theophylline clearance, such as macrolide antibiotics, cimetidine, oral agents, oral contraceptives, leukotriene synthesis inhibitor, and ciprofloxacin. Theophylline overdosage and elevated theophylline levels have been associated with headaches, vomiting, cardiac arrhythmias, seizures, and death. ANTI-IgE (OMALIZUMAB). Omalizumab is a humanized monoclonal antibody that binds IgE, thereby preventing its binding to the high-affinity IgE receptor and blocking IgE-mediated allergic responses and inflammation. Since it is unable to bind IgE that is already bound to high- affinity IgE receptors, the risk of anaphylaxis via direct IgE cross linking by the drug is circumvented. It is FDA-approved for patients >12 yr old with moderate to severe asthma, documented hypersensitivity to a perennial aeroallergen, and inadequate disease control with inhaled and/or oral corticosteroids. It is given every 2–4 wk subcutaneously based on body weight and serum IgE levels. Its clinical efficacy as an “add-on” therapy for patients with moderate to severe allergic asthma has been demonstrated in large clinical trials, with asthmatics receiving omalizumab having fewer asthma exacerbations and symptoms while reducing their ICS and/or oral corticosteroid doses. It is generally well tolerated, although local injection site reactions can occur. Hypersensitivity reactions (including anaphylaxis) and malignancies have been very rarely associated with Omalizumab use. 10 Quick-Reliever Medications. Quick-relief or “rescue” medications (short-acting inhaled β- agonists, inhaled anticholinergics, and short-course systemic corticosteroids) are used in the management of acute asthma symptoms. Asthma Exacerbation Management (Status Asthmaticus) RISK ASSESSMENT ON ADMISSION Focused history • Onset of current exacerbation

• Frequency and severity of daytime and nighttime symptoms and

activity limitation

• Frequency of rescue bronchodilator use

• Current medications and allergies

• Potential triggers

• History of systemic steroid courses, emergency department visits,

hospitalization, intubation, or life-threatening episodes

Clinical assessment • Physical examination findings: vital signs, breathlessness, air movement, use of accessory muscles, retractions, anxiety level, alteration in mental status

• Pulse oximetry

• Lung function (defer in patients with moderate to severe distress or

history of labile disease)

TREATMENT DRUG AND MECHANISMS OF CAUTIONS AND ADVERSE EFFECTS TRADE NAME ACTION AND DOSING Oxygen (Mask or • Monitor pulse oximetry to maintain nasal cannula) oxygen saturation >92%

• Cardiorespiratory monitoring

Inhaled short- Bronchodilator • Nebulizer:when giving concentrated acting β-agonists forms, dilute with saline to 3 mL total nebulized volume

Albuterol nebulizer Nebulizer: 0.15 mg/kg • For MDI:use spacer/holding chamber solution (5 mg/mL (minimum: 2.5 mg) as often concentrate; 2.5 as every 20 min for 3 doses mg/3 mL, 1.25 mg/3 as needed, then 0.15–0.3 mL, 0.63 mg/3 mL) mg/kg up to 10 mg every 1–4 hr as needed, or up to 0.5 mg/kg/hr by continuous nebulization Albuterol MDI (90 2–8 puffs up to every 20 min • During exacerbations, frequent or

11 DRUG AND MECHANISMS OF CAUTIONS AND ADVERSE EFFECTS TRADE NAME ACTION AND DOSING μg/puff) for 3 doses as needed, then continuous doses can cause pulmonary every 1–4 hr as needed , V/Q mismatch, and hypoxemia

Levalbuterol 0.075 mg/kg (minimum: 1.25 • Adverse effects: palpitations, (Xopenex) nebulizer mg) every 20 min for 3 doses, tachycardia, arrhythmias, tremor, solution (1.25 then 0.075–0.15 mg/kg up to hypoxemia mg/0.5 mL 5 mg every 1–4 hr as needed, concentrate; 0.31 or 0.25 mg/kg/hr by mg/3 mL, 0.63 mg/3 continuous nebulization mL, 1.25 mg/3 mL) Systemic Anti-inflammatory • Levalbuterol 0.63 mg is equivalent to Corticosteroids 1.25 mg of standard albuterol for both efficacy and side effects.

Prednisone 0.5–1 mg/kg every 6–12 hr • If exposed to chickenpox or measles, for 48 hr, then 1–2 mg/kg/day consider passive immunoglobulin bid (maximum: 60 mg/day) prophylaxis. Also, risk of complications with herpes simplex and tuberculosis.

1,2.5,5,10,20,50 mg • For daily dosing, 8 AM administration tablets minimizes adrenal suppression

Methylprednisolone • Children may benefit from tapering if

(Medrol) course exceeds 7 days

2,4,8,16,24,32 mg • Adverse effects monitoring: Frequent tablets; bursts risk numerous corticosteroid adverse effects (see Chapter 579 ). See Table 143-13 for adverse effects screening recommendations

Prednisolone 5 mg tablets; 5 mg/5 mL and 15 mg/5 mL solution Depo-Medrol (IM); Short-course “burst” for Solu-Medrol (IV) exacerbation: 1–2 mg/kg/day qd or bid for 3–7 days Anticholinergics Mucolytic/bronchodilator • Should not be used as first-line therapy;

added to β2-agonist therapy

Ipratropium Atrovent (nebulizer Nebulizer: 0.5 mg q6–8 hr • Nebulizer:may mix ipratropium with solution 0.5 mg/2.5 (tid-qid) as needed albuterol mL;MDI 18 μg/inhalation) MDI:2 puffs qid 12 DRUG AND MECHANISMS OF CAUTIONS AND ADVERSE EFFECTS TRADE NAME ACTION AND DOSING Ipratropium with albuterol DuoNeb nebulizer 1 vial by nebulizer qid solution (0.5 mg ipratropium + 2.5 mg albuterol/3 mL vial) Injectable Bronchodilator • For extreme circumstances (e.g., Sympathomimetic impending respiratory failure despite

high-dose inhaled SABA, respiratory failure)

Epinephrine Adrenalin 1 mg/mL SC or IM:0.01 mg/kg (max (1 : 1000) dose 0.5 mg); may repeat after 15–30 min EpiPen autoinjection device (0.3 mg; EpiPen Jr 0.15 mg) Terbutaline Continuous IV infusion • Terbutaline is β-agonist-selective

(terbutaline only): 2–10 relative to epinephrine μg/kg loading dose, followed by Brethine 1 mg/mL • Monitoring with • Adverse effects: tremor, tachycardia, continuous infusion: palpitations, arrhythmia, hypertension,

cardiorespiratory headaches, nervousness, nausea, monitor, pulse 0.1– vomiting, hypoxemia 0.4 μg/kg/min. Titrate in 0.1–0.2

μg/kg/min oximetry, blood pressure, serum potassium increments every 30 min, depending on clinical response.

RISK ASSESSMENT FOR DISCHARGE Medical Stability Discharge to home if sustained improvement in symptoms and bronchodilator treatments are at least 3 hr apart, normal physical findings, PEF > 70% of predicted or personal best, oxygen saturation > 92% on room air 13 DRUG AND MECHANISMS OF CAUTIONS AND ADVERSE EFFECTS TRADE NAME ACTION AND DOSING Home Supervision Capability to administer intervention, and to observe and respond appropriately to clinical deterioration IM, intramuscular; MDI, metered dose inhaler; PEF, peak expiratory flow; SABA, short-acting beta- agonist;SC, subcutaneous; V/Q, ventilation-perfusion.

SHORT-ACTING INHALED β-AGONISTS (SABA). Given their rapid onset of action, effectiveness, and 4–6 hr duration of action, SABAs (albuterol, levalbuterol, terbutaline, pirbuterol) are the first drugs of choice for acute asthma symptoms (“rescue” medication) and for preventing exercise-induced bronchospasm. β-Agonists bronchodilate by inducing airway smooth muscle relaxation, reducing vascular permeability, reducing airways edema, and improving mucociliary clearance. Levalbuterol, or the R-isomer of albuterol, has less tachycardia and tremor, which can be bothersome to some asthmatics. Overuse of β-agonists is associated with an increased risk of death or near-death episodes from asthma. This is a major concern for some patients with asthma who rely on the frequent use of SABAs as a “quick fix” for their asthma, rather than using controller medications in a preventive manner. It is helpful to monitor the frequency of SABA use, in that use of at least 1 MDI/mo or at least 3 MDIs/year (200 inhalations/MDI) indicates inadequate asthma control and necessitates improving other aspects of asthma therapy and management. ANTICHOLINERGIC AGENTS. As bronchodilators, the anticholinergic agents (ipratropium bromide) are much less potent than the β-agonists. Inhaled ipratropium is primarily used in the treatment of acute severe asthma. When used in combination with albuterol, ipratropium can improve lung function and reduce the rate of hospitalization in children who present to the emergency department with acute asthma. Ipratropium is the anticholinergic formulation of choice for children because it has few central nervous system adverse effects and it is available in both MDI and nebulizer formulations. Although widely used in children with asthma exacerbations of all ages, it is approved by the FDA for children >12 yr of age.

A severe exacerbation of asthma that does not improve with standard therapy is termed status asthmaticus. Immediate management of an asthma exacerbation involves a rapid evaluation of the severity of obstruction and assessment of risk for further clinical deterioration. For most patients, exacerbations will improve with frequent bronchodilator treatments and a systemic corticosteroid course. The optimal management of a child with an asthma exacerbation should include, however, a more comprehensive assessment of the events leading up to the exacerbation and the underlying disease severity. Indeed, the frequency and severity of asthma exacerbations helps to define the severity of a patient's asthma. Whereas most children who experience life-threatening asthma episodes have moderate to severe asthma by other criteria, some children with asthma appear to have mild disease except when they suffer severe, even near-fatal exacerbations. The biological, environmental, economic, and psychosocial risk factors associated with asthma morbidity and death can further guide this assessment.

PROGNOSIS. Recurrent coughing and wheezing occurs in 35% of pre–school-age children. Of these, ⅓ continue to have persistent asthma into later childhood, while ⅔ improve on their own through the preteen years. Asthma severity by the ages of 7–10 yr of age is predictive of asthma persistence in adulthood. Children with moderate to severe asthma and with lower lung function measures are likely to have persistent asthma as adults. Children with milder asthma and normal lung function are likely to improve over time, with some becoming periodic (disease-free mo to yr); however, complete remission for 5 yr in childhood is uncommon.

14 Question: 1 The mother of a child who has a history of asthma and sinusitis calls you in the middle of the night because the boy is coughing incessantly. She states that once previously you had prescribed a very effective cough suppressant, and she would like another prescription now. You explain to her that the proper use of a cough suppressant in her child is to treat cough due to A. a viral syndrome B. an asthma exacerbation C. bacterial pneumonia D. postnasal drip from acute sinusitis E. psychogenic cough

Question: 2 You are reviewing results of the pulmonary function tests of a new patient who was recently hospitalized for respiratory problems. You note that the child has a restrictive pattern on testing. Because the cause of a restrictive pattern for most children is poor technique, you review his other pulmonary function tests, which reveal that the pattern is reproducible. Of the following, the MOST likely disease for this child is A. allergic rhinitis B. asthma C. Becker type muscular dystrophy D. chronic bronchitis E. vocal cord dysfunction

Question: 3 A 6-year-old girl has been admitted to the pediatric intensive care unit (PICU) after a near-drowning incident. She received immediate cardiopulmonary resuscitation at the scene and was intubated by the paramedics. In the PICU, she requires 80% inspired oxygen and peak end-expiratory pressures of 12 cm H2O to keep her oxygen saturations above 95%. Chest radiography shows diffuse bilateral pulmonary infiltrates. Of the following, the MOST likely late sequela of her condition is A. asthma B. bronchiectasis C. emphysema D. pulmonary embolus E. pulmonary fibrosis

Question: 4 A 14-year-old child presents for a routine health supervision visit. During the evaluation, he reveals that he has trouble running and that he wheezes even during short-term exercise. He also occasionally wheezes when he has a cold or goes out into cold air. As you review his chart, you note that he was diagnosed with asthma as a young child, but he did not receive any therapy from 5 to 12 years of age. Two years ago, he was diagnosed with exercise-induced asthma and was prescribed and uses a beta agonist inhaler. Of the following, the BEST next step would be to A. order chest radiography B. order pulmonary function testing C. prescribe controller therapy D. refer him to a pulmonologist E. use a spacer with his inhaler

Question: 5 During attending rounds for pediatric residents at the local children's hospital, the team is discussing a patient who recently has been diagnosed as having asthma. As you review the therapeutic options, one 15 of the residents asks about the drug theophylline. You state that the drug is not used frequently at this time. The PRIMARY reason for the decreased use of theophylline is that A. it has a narrow therapeutic index B. it is less effective in suspension form C. it is not effective therapy D. patients do not like to take pills E. therapeutic levels are difficult to maintain

Question: 6 The parents of a 3-year-old boy ask if their son has asthma and if so, what are the chances that he will outgrow it. You explain that the diagnosis of asthma is not always clear-cut, but his respiratory symptoms with activity, weather changes, and viral infections coupled with his allergy to dust mites and cats suggests that he probably does have asthma. Of the following, the MOST appropriate response to their question about their son outgrowing asthma is that A. allergies do not play a role, and he has a 75% chance of outgrowing it B. because he has allergies, he has a less than 50% chance of outgrowing the asthma C. he has a 95% chance of outgrowing it, but not until middle age D. he will not outgrow his asthma because he has allergies E. you cannot predict if he will outgrow his asthma

Question: 7 At your weekly journal club, you are discussing the proper role of leukotriene antagonists in the treatment of the pediatric patient who has asthma. The discussion centers on the severity of the disease and the role of the drugs as add-on therapy to inhaled steroids. The National Heart, Lung and Blood Institute (NHLBI) has issued guidelines for leukotriene antagonist use. Of the following, the MOST appropriate use of leukotriene antagonists, according to NHLBI guidelines, is for A. allergic rhinitis B. mild intermittent asthma C. mild persistent asthma D. moderate persistent asthma E. severe persistent asthma

Question: 8 An 11-year-old boy has a history of a cough over the past two winters that was caused by sinusitis. He also coughed as a young child due to asthma. He presents today complaining of a cough that lasts all day and is loud and disturbing. Upon questioning, the mother states that he does not cough at night. Of the following, the MOST likely diagnosis is A. allergic rhinitis B. cystic fibrosis C. psychogenic cough D. reactive airway disease E. sinusitis

Question: 9 Your practice is creating an asthma program. During the discussion about whether spirometry should be included, one of your associates asks what spirometry measures. Of the following, the BEST explanation of what spirometry measures is 16 A. absolute lung volume B. diffusion capacity C. exhaled nitric oxide D. forced expiratory volume E. percent saturation of blood gases

Question: 10 You are evaluating a 5-year-old boy for moderate reactive airway disease. You explain to the parents that the best form of therapy is an inhaled corticosteroid, but the parents are resistant to this approach. Of the following, your BEST response would be to A. explain that the benefits outweigh the risks and that the inhaled corticosteroid should be used B. prescribe an antileukotriene and a sodium cromolyn preparation in place of the inhaled corticosteroid C. prescribe an antileukotriene in place of the inhaled corticosteroid D. prescribe an inhaled corticosteroid at half the recommended dosage to decrease adverse effects E. prescribe chronic inhaled beta agonists for the next 2 months and re-evaluate the child if he still has moderate asthma

Question: 11 During a routine health maintenance visit for an 8-year-old child, you ask about his asthma. He states that he rarely uses his rescue beta agonist. His mother comments that he 3 nights a week. He reports some chest tightness when he plays soccer on the weekend, but it goes away if he sits out the rest of the game. Of the following, the MOST appropriate therapeutic intervention is A. beta agonists before and during sports only B. exercise challenge to evaluate the degree of his asthma with sports C. long-term controller therapy D. methacholine challenge to evaluate the severity of his asthma E. twice-daily treatment with a long-acting beta agonist

BIBLIOGRAPHY

BASIC 1. Behrman Richard E., Robert M. Kliegman, Hal B. Jenson, eds. Nelson Texbook of Paediatrics. Philadelfia: WB Saunders, 18th edition - 2014. – 994 р. – Р.638. 2. Buryak V.N., Makhmutov R.F., Poshehonova J.V. Children`s diseases. – Donetsk: Nord-Press, 2010. – 328 p. 3. Pediatry. Guidance aid / Ed. by O. Tiazhka – Kyiv: Medicine, 2007. 4. Рediatrics: textbook/ T.O. Kryuchko, O.Y. Abaturov, T.V. Kushnereva, at all. Edited by T.O. Kryuchko, O.Y. Abaturov. Kyiv: AUS Medicine Publishing, 2016. - 208 p.+ 2p. of color insert.

ADDITION 1. Global Strategy for Asthma Management and Prevention, 2015 // The GINA reports are available on www.ginasthma.org 2. Upper Respiratory Tract Infection // Anne Meneghetti, MD, Assistant Professor of Medicine, Tufts University School of Medicine; Medical Broadcaster, Life, Love and Health Contributor Information and Disclosures Updated: Aug 12, 2009. 3. Illustrated Textbook of Paediatrics/ Ed.: T. Lissauer, G. Clayden.- Edinburg: Mosby, 2006.- 410 pp. 4. Forfar & Arneil’s Textbook of Pediatrics/ Ed.: N.Mc Intosh, P.J. Helms, R.L. Smyth, S. Logan.- Edinburg: Churchill Livingstone, 2008. - 1687 pp.

17 THEMATIC SITES: http://www.freebookcentre.net/medical_text_books_journals/pediatrics_online_texts_download.html http://pediatrics.aappublications.org http://www.generalpediatrics.com http://www.aap.org/en-us/Pages/Default.aspx http://hospitalpediatrics.org

Methodical instructions were discussed and approved at the meeting of department of Pediatrics №2 protocol № ___ of ______20____.

Additions and changes were made “____” ______20____ Additions and changes were made “____” ______20____ Additions and changes were made “____” ______20____ Additions and changes were made “____” ______20____

The methodical instructions are compiled by Associate Prof Poda O.A.

Methodical instructions were discussed and approved at the meeting of department of Pediatrics №2 protocol № ___ of ______20____. Additions and changes were made “____” ______20____

Additions and changes were made “____” ______20____ Additions and changes were made “____” ______20____ Additions and changes were made “____” ______20____ Additions and changes were made “____” ______20____

18 MINISTRY OF HEALTH OF UKRAINE UKRAINIAN MEDICAL STOMATOLOGICAL ACADEMY DEPARTMENT OF PEDIATRICS №2

Approved at the meeting of department of Pediatrics №2 Minutes № 21 " 05 " 06 2020 Head of the department MD, Prof. Kryuchko T.O. ______

METHODICAL INSTRUCTIONS FOR INDEPENDENT WORK OF STUDENTS DURING THE TRAINING FOR THE PRACTICAL (SEMINARS) CLASS

Academic discipline Pediatrics Моdule № 1 Content mоdule № 2 The topic of the studies The most common congenital heart diseases in children.

Course (year) 4 Faculty Medical

Poltava 2020 1. Actuality of the topic. Congenital heart disorders (CHD) are the most widespread defects of development, they take 30% from the general number of all defects. According to the WOHP, among the newborns number of children with CHD is close to 1%. Every year in Ukraine 4,5-6 thousand of children are born with heart pathology. Very important point concern in time diagnostic of the pathology and correct surgical treatment. If the last one isn’t provided, on the 1 year of life dose to 55% of children with CHD, and 85% of children before 5 years of age will die. So, severe course of the disease, appearance of unreversable complications, that invalidize child, high mortality rate es- timate actuality of the early diagnostic of CHD, exact topical diagnostic and in time correct sur- gical treatment.

2. The specific aims: 1. To estimate etiological reasons and haemodynamics in case of the most widespread congenital heart disorders (CHD) in children with defects enlarging of pulmonary blood circula- tion (Patent ductus arteriosus (PDA), Ventricular septal defect (VSD), Atrial septal defect (ASD), and defects with normal pulmonary blood circulation (aortal stenosis, aortal coarcta- tion, stenosis of pulmonary artery) and defects with decreased pulmonary blood circulation (Defects of Fallot, transposition of great vessels). 2. To classify and analyse typical clinical changes of the most widespread CHD in chil- dren. 3. To create plan of examination and analyse results of laboratory and instrumental explo- rations in case of typical course of the most widespread CHD in children. 4. To demonstrate skills of main treatment principles, rehabilitation and prevention of the most widespread CHD in children. 5. To put previous diagnos in case of the most widespread CHD in children. 6. To make prognosis in case of the most widespread congenital heart disorders in chil- dren. 7. To demonstrate skills of moral and deontological principles of medical stuff and prin- ciples of subordination in cardiochemotherapy in childhood period.

3. Basic knowledge, skills, habits necessary for studing of the topic (inter-disciplinary inte- gration) The names of the preceding dis- The acquired skills ciplines 1. Normal human anatomy To know main anatomical peculiarities of cardial system in children at different periods of their development. 2. Pathological physiology To know main physiological and pathophysiological peculi- arities of cardial system in children in different ontogenetic periods. 3. Pharmacology To know main groups of medicines that are used in treat- ment of CHD. 4. Public health To use knowledge concerning structure of medical –sanitary help to children population with the aim of adequate using of public health ability in points of prevention and treatment of CHD. 5. Care of the children and nurs- To demonstrate skills of care after the children with CHD ing practice and giving of first medical help. 6. Propadeutic pediatry To estimate main clinical symptoms of CHD in children, to know supplemental methods of examination used for the diagnostic.

4. The tasks for independent work during the training for the studies. 4.1. The list of basic terms, parameters, characteristics, which the student should master while training for the studies:

Definition Meaning Defects with enlarged pul- In this group disorder of haemodynamic, explained by patho- monary blood circulation logical connections between heart cameras and magistral ves- sels, is observed. Defects with normal pulmo- Problem is connected with presence barriers on way of back nary blood circulation circulation such as narrowing. 3). Patent ductus arteriosus Persistance of arterial ductus, after the passing of period of its (PDA) closing and what leads to the haemodynamic disorders. Ventricular septal defect Defect that makes overloading of small circulation circle and (VSD) volume overwork of left ventricle. Atrial septal defect (ASD) Group of CHD that characterized with communication between atriums. Aortal stenosis CHD that is characterized with aortal stenosis (valvular, su- praalveolar or undervulvular narrowing) Aortal coarctation Narrowing or full interruption of aorta in region of aortal cervi- cal part in border of arc and descending parts. Stenosis of pulmonary ar- CHD that means narrowing of pulmonary arteria or taking our tery system of right ventricle. Defects with decreased pul- This group of disorder has ability of venosus-arterial throwing monary blood circulation down, decreasing of pulmonary blood circulation, decreasing of oxygen concentration in arterial blood. Defects of Fallot On the base of pathological anatomy of Falot defect there are 4 components: stenosis or atresia of leading out part of the right atrium, large defect of ventricular septum, hypertrophy of right ventricular myocard and dextraposition of aorta. Transposition of great vesels Aorta is coming from the right ventricle, pulmonary artery from the left.

4.2. The theoretical questions to the studies: 1). Etiology of CHD. 2). Meaning of intranatal injections in developing of CHD. 3). Classification of CHD. 4). Clinical-instrumental diagnostic peculiarities of defects with enlarging pulmonary blood circulation. 5). Clinical-instrumental diagnostic peculiarities of defects with normal pulmonary blood circulation. 6). Indications for the children’s sending to special cardiosurgical clinics. 7). Complications and supervised diseases that can appear in case of CHD. 8). Therapy of emergency conditions (heart failure). 9). Dyspanser observance of children with CHD. 10). Prognosis in case of CHD. 11). Peculiarities of CHD prevention.

4.3. The practical works (tasks) which are done at the studies: 1. Work with tests. 2. Students work in wards near the bed of the patients. 3. To diagnost CHD: defects enlarging of pulmonary blood circulation (Patent ductus arteriosus (PDA), Ventricular septal defect (VSD), Atrial septal defect (ASD) and defects with normal pulmonary blood circulation (aortal stenosis, aortal coarctation, stenosis of pul- monary artery) and defects with decreased pulmonary blood circulation (Defects of Fallot, transposition of great vessels). 4. To indicate conditions, that make danger for the life of the child. 5. To create plan of examination for children with CHD. 6. To prescribe adequate treatment and give emergency help in case of complications ap- pearance. 7. Clinical discussion of presentable situation. 8. Solving of situation tasks.

5. The contents of the topic:

Content of the topic – is full presented in the next resources: lectures, educational and scientific literature from the topic.

Atrial Septal Defects Atrial septal defects (ASDs) comprise 7% to 10% of CHD. Based on their embryological origin, they can be classified as secundum ASDs (the most common type, due to a deficiency in the cen- tral part of the septum primum that develops from the middle of the atrial cavity and grows infe- riorly to meet the endocardial cushion), primum ASDs (due to deficient proliferation of the en- docardial cushion), sinus venosus ASDs (defects near the superior vena cava and right upper pulmonary vein, truly a form of partial anomalous pulmonary venous drainage), and other rarer forms of ASDs. Shunting of blood from left to right at the atrial level leads to increased diastolic blood volume in the RV, which causes right-sided chamber dilation. Clinical Manifestations and Diagnosis. ASDs are typically discovered when a murmur is heard at a regular 4- to 6-month infant well- child visit. The murmur is loudest over the pulmonic region and is associated with a fixed split- ting of the S2 during different phases of respiration and a loud S1. The murmur heard is from in- creased blood flow across the pulmonary valve because of the greater volume of blood in the right side of the heart (relative pulmonic stenosis). An additional diastolic murmur may be heard at the left lower sternal border from excess flow across the tricuspid valve. There may be left precordial bulging due to the enlarged RV being present during cartilaginous rib development. Patients with ASDs are usually asymptomatic but may have some fatigue. They are followed up with Doppler echocardiography to monitor enlargement of the right atrium and/or RV, which, if present, indicates hemodynamic significance. Infants with an ASD manifesting with features of CHF (tachypnea, increased work of breathing, frequent pauses in feeding, failure to thrive, pro- fuse sweating while feeding, decreased activity levels) must be investigated for an additional le- sion such as a PDA, VSD with pulmonary arterial stenosis, or a left-sided obstructive lesion. Ventricular Septal Defects VSDs are the most common CHD lesion and are present in 50% to 60% of all children with CHD. VSDs develop from defective formation of the interventricular septum and are classi- fied on the basis of their location in the septum relative to the atrioventricular valves and the right and left ventricular outflow tracts. Perimembranous VSDs (deficiency in a fibrous part of the septum at the base of the heart) are the most common type, constituting about 80% of all VSDs, followed by doubly committed juxta-arterial (deficiency in the infundibular septum, be- tween the pulmonary and aortic valves), muscular (in the trabecular part of the septum), and inlet (deficiency in the septum inferior to the perimembranous region and above the level of cordal attachments of the atrioventricular valves). Size of the VSD is defined relative to the area of the aortic valve: small (less than one-third of the area), moderate (one-third to two-thirds of the ar- ea), and large (more than two-thirds of the area). Clinical Manifestations and Diagnosis. A newborn with a VSD may not initially have a murmur; however, as the pulmonary re- sistance decreases with age, an S1-coincident pansystolic murmur can be heard the loudest over the left lower sternal border. A diastolic rumble at the apex may be heard from excess flow across the mitral valve. A child with a hemo-dynamically significant VSD presents with features of pulmonary overcirculation and CHF. A chest radiograph shows cardiomegaly with pulmonary vascular congestion, while EKG shows left or biventricular hypertrophy. As the child grows, some perimembranous VSDs can get occluded by aneurysmal tissue, and muscular VSDs can become smaller in size with muscular growth. One must be wary of the perimembranous defect that becomes smaller owing to prolapse of the aortic valve. Patent Ductus Arteriosus An arterial duct is a normal fetal connection between the aorta and the pulmonary artery that is present in all newborns. It closes functionally within 24 hours and anatomically within 3 to 4 weeks in most patients. It may remain patent longer in some patients, especially infants born prematurely. Clinical Manifestations and Diagnosis. The examination of patients with PDA varies with their age. In a newborn with higher pulmonary resistance and pressures, the PDA may not shunt much blood and may not be audible. In an older infant, however, one can detect a loud systolic or continuous murmur of blood shunt- ing left to right during the entire cardiac cycle, which is loudest over the left precordium. In an older patient with a hemodynamically significant PDA, one may be able to detect a diastolic rumble of transmitral flow into an enlarged left ventricle (LV). The left-to-right shunting caused by the PDA may also lead to features of CHF. Chest radiographs illustrate features of pulmonary overcirculation and cardiac enlargement, EKG can show LV hypertrophy and ST-segment changes of ischemia (beware of this finding in a newborn, as it implies a very large shunt), and Doppler echocardiograms can demonstrate left atrium and LV enlargement, characteristics of flow, and anatomy of the PDA. Management. Ibuprofen and indomethacin have been used to medically close PDAs in premature in- fants. Acetaminophen is also being studied as an alternative medical therapy. A symptomatic or hemodynamically significant PDA is a class I indication for intervention, with either device oc- clusion in the interventional catheterization laboratory or surgical ligation. In a center with inter- ventional cardiac catheterization expertise, device occlusion is the first-line approach for an older infant or child, with excellent results and extremely low complication rates comparable to those of surgical ligation. In babies with low birth weight and those with extremely low birth weight, personnel at some centers lean toward surgical ligation, often at the bedside. However, success- ful PDA device occlusion has been reported in small neonates, and the most recent series demonstrates successful catheter-based intervention in neonates as small as 700 g. Both proce- dures have similarly high success rates and a complication rate of less than 1%. Patients with left-to-right shunt lesions (eg, with a VSD, AVSD, PDA, and, in very rare instances, an ASD) that present late may have pulmonary hypertension. The degree of pulmonary hypertension due to pulmonary vascular obstructive disease may be prohibitive for repair. Some of these patients will be treated with agents that lower pulmonary hypertension and may later in life go on to de- velop Eisenmenger syndrome (when pulmonary vascular resistance exceeds systemic vascular resistance, resulting in a reversal of the shunt) and its associated sequelae. CoA and Interruption of the Aorta CoA is a narrowing at the isthmus of the arch of the aorta (with concomitant narrowing of the transverse arch in some cases), constituting 5% to 8% of CHD. IAA is the most extreme end of this spectrum, with a discontinuation of the arch and distal continuation through a PDA past the point of interruption. IAA accounts for about 1.5% of CHD and is classified according to the site of the interruption: type A is interrupted at the isthmus distal to the left subclavian artery, type B is interrupted between the origins of the left subclavian and left common carotid arteries (the most common type and frequently associated with chromosome 22 abnormalities), and type C is interrupted between the origins of the innominate and left common carotid arteries (the most rare type). About 80% of patients with IAA have an associated VSD. Clinical Manifestations and Diagnosis. Severe and critical CoA and IAA may be detected at the time of newborn screening with a lower saturation level at the postductal site or via clinical suspicion on the basis of poor pedal or femoral pulses at the first newborn visit to the pediatrician. These patients may present in ex- tremis at 2 to 3 weeks of age, in shock, with feeble pulses, lethargy, poor feeding, decreased urine output, and metabolic acidosis. While sepsis remains the most common cause of this con- stellation of symptoms, an echocardiogram should be performed for CoA and/or IAA if the shock is unresponsive to fluid resuscitation. Mild CoA may be detected on the basis of a gradient of more than 20 mm Hg between the upper- and lower-extremity blood pressures (carefully per- formed with proper technique) during infancy. A harsh systolic murmur that is loudest over the back may be heard. CoA may also be found in young adolescents undergoing workup for hyper- tension. There may not be a clinically significant blood pressure gradient in older patients with CoA because they develop collateral vessels over time that supply blood distal to the narrowing. In the case of collateralization, one can detect a continuous murmur of the flow in these vessels over the chest wall. Management. Newborns with severe or critical CoA and IAA are dependent on prostaglandin infusion to keep the PDA open until the time of surgical repair. Some children (particularly older chil- dren) are candidates for intervention in the cardiac catheterization laboratory, with balloon angi- oplasty and stent placement for the coarctation. After repair, patients are followed up closely in the cardiology clinic to monitor for recurrence of CoA and persistent hypertension and continue to receive long-term follow-up at 1–2-year intervals. Tetralogy of Fallot сomponents: - High VSD - Pulmonic stenosis, i.e. right ventricular outflow obstruction usually infundibular, sometimes valvular - Overriding of the aorta - Right ventricular hypertrophy Tetralogy of Fallot is the most common cyanotic CHD, accounting for 5% of all CHD. It develops from anterior malalignment of the interventricular septum, which leads to a VSD, as well as overriding of the VSD by the aorta. There is a narrowing of the pulmonary outflow tract due to the septal deviation, and this causes RV outflow (infundibular) obstruction and conse- quent RV hypertrophy. Critical Component: Degree of pulmonic stenosis • Regulates degree of R « L shunt • Regulates overriding of aorta • Greater the stenosis, the greater the aortic overriding Clinical Manifestations and Diagnosis. Patients with tetralogy of Fallot have a harsh ejection systolic murmur heard over the pulmonic area, indicating pulmonic stenosis. There may be a single second sound. Higher satura- tions indicate less RV outflow obstruction. Patients who do not receive a diagnosis prenatally may present for the first time as children having a hypercyanotic spell in a period of agitation, fever, or other concurrent illness. Agitation and crying increase pulmonary vascular resistance, while also increasing the heart rate. Owing to a subsequently shorter diastolic period, ventricular filling is less, which adds to the obstruction to the RV outflow from its hypertrophic muscle bun- dles. “Tet spells” may become a vicious cycle and require a combination of heart rate control and vascular resistance manipulation to break. The murmur during a hypercyanotic spell or “Tet spell” becomes softer because of decreased pulmonary flow. Iron deficiency anemia will accel- erate the onset of these spells. Clinical findings: Squatting Dyspnea Failure to thrive Cyanosis-usually Severe cases ↔ at birth ↔ severe PS Mild cases ↔ much later ↔ mild PS “Pink tets” (acyanotic) and “Blue tets” (cyanotic) Imaging Findings: Heart size normal • Rarely enlarged • Cardiac apex displaced upward “coer en sabot” • PA segment concave • Decreased vasculature • R aortic arch in 25% Management. The time of presentation and intervention for tetralogy of Fallot is determined by the degree of RV outflow obstruction and the limitation of pulmonary blood flow. These clinical indicators are the oxygen saturation levels and the development of hypercyanotic spells. The Doppler echocar- diographic indicators are the pressure gradients observed across the RV outflow tract. Children with saturations less than 80% or those having hypercyanotic spells are scheduled for surgery. Acute “Tet spells” are managed by first helping to calm the patient (eg, handing the child to the parent to hold, allowing the child to feed, or administering sedation if needed), placing the child in a knees-to-chest position (to increase systemic vascular resistance; this may be achieved by having the parent hold the baby in her arms and cradling the knees and chest together), initiating oxygen (preferably through the least noxious route for the child, for example, blow-by oxygen or use of a face mask), administering a bolus of intravenous fluids, and, finally, intravenous metoprolol (to slow the heart rate) and phenylephrine (to increase systemic vascular resistance). Some patients will require anesthesia (on the way to the operating room). Surgical management includes palliative procedures like a modified Blalock-Taussig-Thomas shunt to help provide consistent pulmonary blood flow in the setting of significant stenosis or atresia of the pulmonary valve. Complete repair of the heart comprises closure of the VSD, as well as resection of the RV obstruction, resulting in normal saturations. Complete repair is replacing the palliative approach in many centers. Even after complete tetralogy of Fallot repair, patients require lifelong cardiol- ogy follow-up, since their pulmonary valve may become more regurgitant (depending on the surgical approach and the intervention on the valve annulus itself), which can lead to RV dila- tion. Patients may also develop arrhythmias because of the RV dilation and/or hypertrophy and scarring (as a result of the pulmonary valve regurgitation, as well as the primary surgery), with an increase in risk of sudden cardiac death in pediatric patients with QRS duration longer than 170 ms on the EKG. Cardiac magnetic resonance imaging is used to estimate the volume of the RV to determine the time for pulmonary valve replacement (catheter-based or surgical). While complete repair of tetralogy of Fallot ensures that these children have normal saturations, most patients have a prolonged QRS duration and features of right bundle block on the EKG, owing to the intervention on the interventricular septum. Transposition of the Great Arteries Transposition of the great arteries is the second most common cyanotic CHD, accounting for about 2% of all CHD. It is the most common cyanotic heart disease manifesting in the first week after birth. There is ventriculoarterial discordance, with the aorta arising from the RV (usu- ally anterior to the pulmonary artery) and the pulmonary artery arising from the LV. Hence, the systemic and pulmonary circulations are in parallel, with systemic venous (deoxygenated) blood returning to the right atrium, the RV, and going out the aorta again. There is mixing of blood at the atrial level through a patent foramen ovale or an ASD or at the ventricular level through a VSD (35% to 40% of transposition of the great arteries). Clinical Manifestations and Diagnosis. Newborns with transposition of the great arteries present with cyanosis within the first 12 hours after birth and are not responsive to oxygen or mechanical ventilation. The presence of a VSD may delay presentation. Chest radiography may show a narrow mediastinal silhouette due to the orientation of the great vessels and thymic regression, and EKG results may be normal or show RV hypertrophy. There is usually no murmur at examination; however, there may be a sin- gle S2 with a loud aortic component due to the orientation of the great vessels. Management. Once the diagnosis is established, these patients require reparative surgery to switch the great vessels to the appropriate ventricles, known as the arterial switch procedure. They may need respiratory support with oxygen, mechanical ventilation, and initiation of prostaglandin E1 until the time of surgery. Prostaglandin allows the PDA to remain open, which shunts blood from the aorta into the pulmonary circulation. This increases the amount of blood returning to the left atrium and mixing at the atrial level, as long as the foramen is open. Most patients undergo a catheter-based procedure called balloon atrial septostomy to help create or enlarge the ASD to allow more mixing while awaiting surgery. If cyanosis persists despite an open PDA, a fluid bo- lus or inotropic support may help improve mixing and systemic arterial saturations. After repair, these patients require regular cardiology follow-up for life. The first arterial switch procedure was performed in 1975, and since the patients undergoing this procedure are followed up for progressively longer periods of time, data about the long-term complications are emerging. De- pending on the study, 5% to 30% of these patients have been reported to require reintervention at 25-year follow-up for a variety of reasons, most commonly regurgitation of the neoaortic valve (more than 75%), supravalvar pulmonary stenosis (more than 75%), and coronary artery disease (5% to 8%).

6. The materials for self-control A. Sample case report Task 1. Child of 4 months of age was found to have tachypnea, periorbital and perioral cyanosis, that becomes more intensive during the cry and eating. After birth cordial murmur was auscultat- ed. During the objective examination: General condition of the child is moderate, t-36,3C. Skin is pale, “marble” skin picture, perioral and per orbital cyanosis. Tachypnea to 50 per mi- nute. Auscultative – rough breathing, dry and moistures whures. Pulse rate is 146 per minute, rhythmical. Apex beat is extensed. Cordial borders are spread to the left. Auscultative – I tone on the apex is cough, II tone is akeented and decomposited on the pulmonary artery, systolic and diastolic murmur at the left subclavicular region. Liver +2cm. On the ECG: hypertrophy of the left ventricularatrium. On the chest X-Ray-cardiomegalie connected with hypertrophy of left ventricle and atrium, intensification of pulmonary picture. On EchoCG – dilatation of the left atrium and ventricle, EE 70%, postcapilar pulmonary hypertension. Tasks: 1.Put clinical diagnose. 2.What haemodynamical changes can explain clinical manifestation of the disease. 3.What treatment principles will be the best in such case? 4.Prescribe recipe on Digoxin for the baby.

Task 2. The child of 3 years of age during the examination of district pediatrician was found to have systolic murmur in the heart. From the anamnesis is known, that murmur was auskultated since the birth. During the objective examination, general condition of the baby is good. Skin and mucous membranes are clean, pink. Breathing is vesicular. PR 95 per minute, rhythmical, resistant on the upper extremitas and thick on the lower. BP on hands 110/85 mm hg, on legs 70/30 mm hg. Apex beat is resistance, localized, is found in 1 cm to left from left linea medi- aclavicularis. Auscultative – accent of the II tone at aorta, moderate intensive systolic murmur on the right border of sternum with irradiation to interscapular region and to the neck vessels. Liver is at the rib’s border. On the ECG – sinosal rhytm, hypertrophy and systolic overload of left ventricle. On the X-Ray- hypertrophy of left ventricle. On EchoCG – hypertrophy of left ventricle walls, signs of left ventricle obstruction. Pressure in the system of pulmonary artery is normal. Tasks: 1.Put clinical diagnose. 2.What haemodynamical changes can explain clinical manifestation of the disease. 3.What treatment principles will be the best in such case? 4.Prescribe recipe on СОRGLYCONE for the baby.

Task 3. The child of 2 months of age is in severe condition. The child is decreased in body weight, has signs of perioral and acrocianosis. Breathing is hard, up to 70 per minute. During the percussion shortering of sound in paravertebral region is found. During the auscultation – lots of crepitative wheezes. Cordial borders are spread to the left and to the right, rough systolic murmur is found at all heart regions, more in III-IV intercostals spaces in left side, accent of the II tone at pulmonary artery. Liver is enlarged on 3 cm, painless. Tasks: 1.Put clinical diagnose. 2.What haemodynamical changes can explain clinical manifestation of the disease. 3.What treatment principles will be the best in such case? 4.Prescribe recipe on STROPHANTHINUM К for the baby.

Task 4. Child is 6 year old; can’t run, quickly becomes tired, often complains on head ache, nasal haemorrhagies. During the examination hypotonia, hypotrophia of legs muscle, paleness of skin an then are found. Upper extremities are developed according to the age. Pulse is strong on radial arteries, pulsation of abdominal and femoral arteries is thick. Blood pressure on the hands 120/80 mm hg, on legs – 60/0 mm hg. During the heart auscultation systolic murmur in the II intercostals space of right and in interscapular region is heard. Tasks: 1.Put clinical diagnose. 2.What haemodynamical changes can explain clinical manifestation of the disease. 3.What treatment principles will be the best in such case? 4.Prescribe recipe on FUROSEMIDЕ for the baby.

Task 5. Born in term child of 8 days of age was found to have rough systolic-diastolic murmur at all heart region. Tasks: 1.Put clinical diagnose. 2.What haemodynamical changes can explain clinical manifestation of the disease. 3.What treatment principles will be the best in such case? 4.Prescribe recipe on RIBOXINE for the baby.

Task 6. Child of 1,5 years of age has usual skin cyanosis since the birth. Delay in physical development, deformations of fingers – “clubbing” and “ watch glasses” were found. During percussion heart borders are moved to the right; during auscultation – the thickness of II tone on the pulmonary artery, rough systolic murmur in III-IV intercostals space in left side. On the chest X-ray enlarg- ing of right cordial parts, poor lung picture. General blood test: Hb-180g/L, erytr. – 5,8x1012/L. Tasks: 1.Put clinical diagnose. 2.What haemodynamical changes can explain clinical manifestation of the disease. 3.What treatment principles will be the best in such case? 4.Prescribe recipe on PROMEDOLUM for the baby.

Task 7. Girl is 11 months old. Parents complain on quick getting tired during feading, increasing of the cyanosis, tachypnea during cry. Objective: general condition of the child is moderate, delay in physical development. In calm condition – moderate cyanosis of the skin, mucous membranes, that increases while the child is crying. During the examination at the backgraound of anxiety, cyanosis became more intensive, tachypnea, child became restless; become more quiet on moth- ers hands with pressed legs to the trunk. Breathing – 64 per minute, under the lungs clear sound is heard during percussion; rough breathing. PR-150 per minute. Heart borders are spread to the right. Rough systolic murmur is heards on all chest region, maximum noisy is on the II inter- costals space left from the sternum, the II tone is thicked at the pulmonary artery. General blood test: Hb-200g/L, erytr. – 6,0x1012/L, CP-0.95, neutroph: s-5%, seg-39%, eos-3%, lymp-50%, mon-3%, SES-4mm/h. Tasks: 1.Put clinical diagnose. What complication began in this situation? 2.What haemodynamical changes can explain clinical manifestation of the disease. 3.What treatment principles will be the best in such case? 4.Prescribe recipe on ANAPRILIN for the baby.

Task 8. To the cardiological depertment Nicolay N. 2,5 years of age was hospitalized. Complications on – cyanotic attacks, child takes forced leg-hand position. From the anamnesis is known, the child is from the 3 pregnancy, the 2 birth. At the 1 pregnancy period mother had ARVI. Delivery in term. Since the birth systolic murmur was auscultated; child was sent for the cordiologis consul- tation. During the objective examination, general condition of the child is moderate. Delay in physical development is found. Moderate skin cyanosis is found. Fingers look like “clubbing” and “ watch glasses” . breathing – 46 per minute. Vesicular breathing is heard under the lung. Defor- mation of the chest- cordial hump, Harrison’s fissure. PR-140 per minute, rhythmical, small vol- ume. Borders of the cordial sound: up –II intercostals space left; left-left line mediaclavicularis, right-right parasternal line. Cordial tones rhythmical, the II tone is increased at pulmonary artery. Rough systolic murmur is heard at all heart region; specially in III intercostals space left. Liver +3cm. General blood test: Hb-186g/L, erytr. – 8,9x1012/L, CP-0.95, neutroph: s-2%, seg-29%, eos-5%, lymp-57%, mon-7%, SES-2mm/h, Ht-0,65. Chest X-Ray-cardiomegalia because of right ventricle hypertrophy, cordial vessels radix is then, pulmonary picture is poor. On EchoCG: dilatation of right ventricle DVS, pressure at pulmonary artery – 65 mm hg. Tasks: 1.Put clinical diagnose. What complication began in this situation? 2.What haemodynamical changes can explain clinical manifestation of the disease. 3.What treatment principles will be the best in such case? 4.Prescribe recipe on ANAPRILIN for the baby.

Task 9. Patient Z., 3 years of age was hospitalized to the cordiological department. From the anamnesis is known, that child after the birth had diffused skin cyanosis. At the age of 7 day was provided with Rashkind (closed atrioseptostomia) procedure. Since the age of 3 month was in the chil- dren’s refused house. Objective: skin and mucouse membranes are cyanotic, acrocyanosis, fingers like “clubbing” and “ watch glasses”, deformation of the chest.Borders of the heart: right – 1cm out from the right parasternal line, left – on left axillar line, up – II rib. Auscultative: tones are rhythmical, PR – 160/min; in the III left intercostals space moderate systolic murmur is heard; accent of the II tone in II intercostals space. Breathing – 40 /min, deep, noisy. Liver + 3cm. General blood test: Hb-146g/L, erytr. – 4,9x1012/L, CP-0.9, neutroph: s-4%, seg-21%, eos-1%, lymp-70%, mon-4%, SES-3mm/h, Ht-0,65. Tasks: 1.Put clinical diagnose. What complication began in this situation? 2.What haemodynamical changes can explain clinical manifestation of the disease. 3.What treatment principles will be the best in such case? 4.Prescribe recipe on NATRII HYDROCARBONAS for the baby.

Task 10. The boy K., 11 month of age, was hospitalized with complications on delay in physical devel- opment (body weight-7kg), appearency of tachypnea and perioral cyanosis in case of physical and emotional work. From the anamnesis is known, that insufficient body weight appeared since 2 months of age. During the feeding quick get tired was observed. Didn’t have bronchitis or pneumonias. Objec- tive: skin is pale with cyanotic tone, symptoms of “clubbing” and “ watch glasses”. Cordial re- gion without changes, heart borders: left – on left mediaclavicular line, right – on right paraster- nal line, up – II intercostals space. Cordial tones without changes, PR – 140 /min. near left ster- num border systolic rough murmur is heard. Breathing is pueril, without wheezes. Lives and spleen are in normal sites. General blood test: Hb-170g/L, erytr. – 5,4x1012/L, CP-0.9, neutroph: s-3%, seg-28%, eos-1%, lymp-64%, mon-6%, SES-3mm/h, Ht-0,65. Tasks: 1.Put clinical diagnose. What complication began in this situation? 2.What haemodynamical changes can explain clinical manifestation of the disease. 3.What treatment principles will be the best in such case? 4.Prescribe recipe on PROMEDOLUM for the baby.

B. The tests for self-control

1. The child is 4 months of age. Development is according to the age. Objective status: the right cordial border of the relative heart dullness is on the right parasternal line; the upper border is on the II rib, the left border is on the distance of 2 cm out of mediaclavicular line. How the explain this result? A. Cardial border are normal age variant. B. Congenital heart disease. C. Cardiomiopathology. D. Hypertrophy. Carditis. E. Pulmonary hypertension.

2. During organization of the child to the children’s group systolic murmur, with maximum sound in II-III intercostals space at the left sternal border, was found. What congenital disorder can be suspected? A. Septal defect. B. Aortal stenosis. C. Coarctation of aorta. D. Fibroelastosis. E. Arterial ductus is opened.

3. The boy at the delivery house was established diagnos: of congenital heart disorders (defect of ventricular septum). At the age of 2 months tachypnoe with frequency 60 per minute, tachycar- dia to 170 strokes per minute, enlarging of liver to 3 cm down from ribs arc appeared. What medicines should be prescribed first of all? A. Cordial . B. Nonsteroidal anti inflammatorial C. Kalium medicines D. β-adrenoblokators E. Glucocorticoides.

4. Patient N., 12 years of age, during the doctor’s examination was found to have hyper devel- opment of upper and underdeveloped low parts of the body. Arterial pressure on the hands is higher than on the legs. During the percussion changes were not found. Systolic murmur is aus- cultated on the back. What diagnos is possible? A.Coarctation of aorta. B. Pulmonary artery stenosis. C. Aortic stenosis. D. Interpulmonary septal defect. E. Ventricular septal defect.

5. During the examination at the hospital boy of 6 months of age was found to have a high ven- tricular septal defect. What part of cardio-vascular system will be overload first of all: A. Right ventricular B. Right atrial C. Left ventricular D. Left atrial E. Large blood circulation.

6. The boy of 10 years of age during the cordial auscultation was found systolic murmur with center in II intercostals space right from the sternum. Murmur good irradiates to the jugular pit region and neck vessels. At the ECG signs of hypertrophy and systolic overload of left ventricu- lar. What’s your diagnos? A. Aortic stenosis. B. Nonrheumatic carditis. C. Coarctation of aorta D. Hypertrophical cardiomyopathia E. Ventricular septal defect.

7. Child of 2 months of age was found that cordial borders are spread to the left, the II bone is intensifical at the pulmonary trunk, without intervals, systolic-diastolic murmur of the machine character at the II-III intercostals space at the left part of sternum, that irradiates to the neck ves- sels. PR 150 b.p.m, AP-105/30 mmhg.rate. on the ECG: signs of hypertrophy at left ventricular. What congenital defect is the most possible? A. Open arterial ductus B. Ventricular septal defect C. Atrial septal defect D. Iezenmenger complex E. Open atrioventricular ductus.

8. The boy of 2 years of age is coming off the physical development, objective status: BR 20 per minute, PR 104 b.p.m, liver + 4cm, spleen – 25[15[10cm. during the auscultation – systolic- diastolic murmur in II-III intercostals space near the sternum, without intervals, lowering of the diastolic arterial pressure. ECG: is without changes. What is your diagnos? A. Open arterial ductus B. Ventricular septal defect C. Atrial septal defect. D. Atresia of threeuvelares valvula. E. Aortic valvular defect.

9. The boy of 5 years of age walks hard quickly becomes tired. Often have a headacke, auricular murmur, blood from nose. Hypotonia and hypotrophia of leg’s muscules; upper part of the body is developed according to the age. Pulse on the radial arteries is strong; pulse on the femoral ar- teries is thick. BP on arms 120/80/ on legs – 60/10 mm hg rate. During the auscultation at the heart- systolic murmur in II intercostals space from right side and in the interscapular region. Previous diagnos: A. Coarctation of aorta B. Defect of Fallot C. Open arterial defects D. Stenosis of pulmonary artery E. Aortal stenosis

10. The boy of 10 years of age during the auscultation was found systolic murmur under the heart region with intensification on the apex. The II tone is forced at the aorta. Systolic murmur is strong auscultated under the left intrascapular space. During the ECG hypertrophy of left ven- tricular. What is your diagnos? A. Coarctation of aorta B. Nonshumatic carditis C. Infectious endocarditis D. Defects of Fallot E. Open arterial ductus.

11.The boy of 3 years of age is observed to have periodical short winding-cyanotical attacks. What is the most possible diagnos? A. Fallo defects B. Myocarditis C. Vegetal-vessels dysfunction, cordial type D. Bronchial astma E. Ventricular septal defect.

12.Which of the compensatory mechanism is impossible in Fallo defects? A. Development of vessels collaterals B. Polycytemia C. Left ventricular hypertrophy D. Lowering of the blood circulation score. E. All pointed.

13.The girl of 4 years of age, has cyanosis from the birth, shortless of breath in calm condition and periodical attacks. Acute underdeveloped in physical growth, cordial hump. Auscultation: rude systolic murmur is heard under the whote cordial surface, that irradiates to the back. ECG: hypertrophy of right ventricular. What cordial disorder is the most possible? A. Fallo defects B. Isolated pulmonary stenosis C. Open arterial ductus. D. Open foramen oval. E. Transposition of magistral vessels.

14.Newborn baby has cyanosis from the birth. On the ECG was found displacement of electrical axis of heart to the left and hypertrophy of left ventricular. What diagnos is the most possible? A.Congenital heart defect. B. Syndrome of breathing disorders. C. Fibroelastosis. D. Small development anomalies. E. Congenital myocarditis.

15.What sign is the most common for shortless of breath-cyanotic attacks: A.Squating, significant intensification of breath insufficiency B.Losting of mind C.Breathing stop. D.Clinical features of lung edema. E.All pointed.

16.Child with Fallo defect during the cyanotic-breathine attack takes forced position- squat, keep knees near the thorax. During this procedure general condition becomes better. What is the most possible reason of the changing of general condition in such position? A.Enlarging of blood circulation through the pulmonary artery. B.Enlarging of blood circulation through the aorta. C.Decreasing of blood circulation through the pulmonary artery. D.Enlarging of the circulation blood volume. E.Decreasing of the circulation blood volume.

17.What are the most common signs of isolated stenosis of pulmonary artery: A.Decoupling of the second tone. B.Rude systolic murmur in II-III intercostals space left from the sternum. C.All pointed is right. D.All pointed is false E.Normal physical development.

18.What are the most common signs of magistral vessels transposition: A.Shortless of breath, cyanosis from the first days of life. B.Thickness of the II tone in the II intercostals space right from the stermum. C.Atrial septal defects. D.Primary bacterial endocarditis. E.Normal physical development.

19.The boy of 4 month of age was found to have progressive skin cyanosis. On the ECG: signs of hypertrophy of right ventricular. Congenital heart disorder is suspected. What diagnostic method is nessesary for diagnos elaboration: A.Echocardiography B.Radiology examination C.Phono cardiography D.Reography E.Polycardiography.

20.What disorder has two isolated blood circulation circuses and supported by other heart de- fects? A.Magistral vessels transpositio B.Atrial septal defect C.Open arterial ductus. D.Fallo defects. E.Atrial stenosis.

7.Literature. Basic: 1.Virgiania A. Keane. Assessment of growth./ in Nelson Textbook of Pediatrics, 2-Volume Set, 20th ed. by Kliegman R.M., Bonita M.D. Stanton MD, Geme J.S., Nina F Schor MD / Elsevier Inc., 2016. 2.Pediatrics: Textbook for students of higher medical educational institutions III-IV levels of ac- creditation. / VG Maidannik. - Kharkov: Folio, 2006. 3. Selected issues of child cardiorheumatology. Manual for students of higher chools IV level of accreditation. / under general ed. prof A.P. Volosovec, V.M. Sava, S.P.Nick. - Kyiv, Kharkiv. - 2006 4. Children's Diseases (V.M Sidelnykov, V. Berezhnaya, B.J. Resnik, etc.). - K.: "Health", 1999. 5. Rheumatology childhood / under general ed. prof. E.V. Prokhorov, A.P. Volosovec.- Donetsk, 2006. 6. Medical childhood / gen.ed.prof. Moschych S.K.: Health, 1994 Additional 1. Derek S. Wheeler, Hector R. Wong and Thomas P. Shanley Cardiovascular Pediatric Critical Illness and Injury, 2nd ed. 2009. 2.Rheumatic Diseases (guide for physicians), ed. VA Nasonova, NV Bunchuk. -M. , Medicine, 1997. 3. Diseases of the heart and vessels in Children .- M.: Medicine, 1987.

The methodical instructions are compiled by Bubyr L.N.

Methodical instructions were discussed and approved at the meeting of department of Pediatrics №2 protocol № ___ of ______20____.

Additions and changes were made “____” ______20____ Additions and changes were made “____” ______20____ Additions and changes were made “____” ______20____ Additions and changes were made “____” ______20____ MINISTRY OF HEALTH OF UKRAINE UKRAINIAN MEDICAL STOMATOLOGICAL ACADEMY DEPARTMENT OF PEDIATRICS №2

Approved at the meeting of department of Pediatrics №2 Minutes № 21 " 05 " 06 2020 Head of the department MD, Prof. Kryuchko T.O. ______

METHODICAL INSTRUCTIONS FOR INDEPENDENT WORK OF STUDENTS DURING THE TRAINING FOR THE PRACTICAL (SEMINARS) CLASS

Academic discipline Pediatrics Моdule № 1 Content mоdule № 2 Acute Rheumatic Fever in children. Juvenile rheumatoid The topic of the studies arthritis and reactive arthritis in children.

Course (year) 4 Faculty Medical

Poltava 2020

1. Actuality of the topic. According to the WHO studying, morbidity on acute rheumatic fever for the last years significantly decreased (from 18,6 on 1000 of pupils in 80th, to 12,8 in 2001). But rheumatic heart defects till nowadays is the main reason of morbidity in youth in case of acute rheumatic fever. From time to time all over the world, also in Ukraine, outbreaks of the disease are observed. That is why ARF still is an actual problem for the most of the doctors all over the world, and also for scientists, interested in this topic. Juvenile rheumatoid arthritis (JRA) is a common, rheumatic disease of children and a major cause of chronic disability. It is characterized by a synovitis of the peripheral joints manifesting in soft tissue swelling and effusion. In the Classification Criteria of the American College of Rheumatology (ACR), JRA is regarded not as a single disease but as a category of diseases with three principal types of onset: oligoarthritis or pauciarticular disease, polyarthritis, and systemic-onset disease. Nine distinct subtypes are identifiable. The European League Against Rheumatism (EULAR) and the International League of Associations for Rheumatology (ILAR) propose an additional classification schema. Only the ACR criteria have been statistically validated by extensive, multi-clinic surveys and meet the requirements of evidence- based medicine.

2. The specific aims: - to know definition of the ARF and rheumatic heart disease, modern etiology of the disease; - to define pathogenesis elements and pathomorphological periods of ARF and their accordance; - to classify and analyse, diagnostic Kisel-Johns criteria of the ARF and analyze clinical picture of heart and out cordial features of the ARF; - to learn how make up the plan of examination and to analyze results of laboratory, instrumental and immunologic investigation; - to carry out differential diagnostics of heart and out cordial disorders in patients with ARF, using results of the objective and laboratory-instrumental, immunologic examinations; - to make a diagnosis according to the ARF classification; - to demonstrate skills of hospital, rest-house, polyclinic stages of the treatment of ARF; - to determine aetiologic and nosotropic factors juvenile rheumatoid arthritis and reactive аrtropaty for children. - to classify and analyse a typical clinical picture juvenile rheumatoid arthritis and reactive аrtropaty for children. - to make the plan of inspection and analyse information of laboratory and instrumental inspections at a typical flow juvenile rheumatoid arthritis and reactive аrtropaty for children. - to formulate a preliminary diagnosis at treatment juvenile rheumatoid arthritis and reactive аrtropaty for children. - to demonstrate the knowledge of deontological principles of a medical worker to the patients with rheumatic diseases, juvenile rheumatoid arthritis and reactive аrtropaty.

3. Basic knowledge and skills, habits necessary for the studying of the topic (interdisciplinary integration) The names of the preceding disciplines The acquired skills 1. Human anatomy To know anatomy of heart, central vessels, structure of large and small joints 2. Pathological anatomy To know main peculiarities of the pathomorphological inflammatory stages 3. Biochemistry To demonstrate knowledge of the points of normal results and changes of general and biochemistry results of the examinations, to explain results of tests on activity of the inflammatory process (seromucoid, CRP) 4. Pharmacology To use skills about the pharmacology of the antibiotics, nonsteriodal anti-inflammatory medicines, glucocorticosteroids, imunnesupresors. To demonstrate skills of the recipes prescribe. 5. Care for children and nurse practice To demonstrate skills of the care for children with heart diseases 6. Propaedeutic pediatric To demonstrate skills of the examination, palpation, percussion, auscultation of the cordial system in case of norm and semiotic of probable disorders. To analyze ECG, EchoCG results 7. Radiology To estimate main X-Ray changes of the joints and heart. 8. Inwardly subject integration To give a clinical estimation the changes of indexes of biochemical research of whey of blood, immunological indexes. 9. Paediatricsof Propaedeutics To own practical skills of inspection of patient, to collection of anamnesis, to have imagination about the additional methods of inspection, that are applied for their diagnostics.

4. The tasks for independent work during the training for the studies. 4.1. The list of basic terms, parameters, characteristics, which the student should master while training for the studies: Systemic disease of the connective tissue with prevalence of the pathological process in cardio- Acute rheumatic fever vascular system that caused by acute streptococcal infection (streptococcus of A group) in inclined persons (7 – 15 years of age) New episode of ARF with manifestation of carditis Repeated rheumatic fever (tricuspid and aortal valvulitis), rarely – carditis and polyarthritis, more rare - chorea Disease characterized with heart valvules injury with Chronical rheumatic heart disease signs of marginal fibrosis of valvular elements or heart disorders, that appeared after ARF Syndrome of ARF with manifestation of migration Polyarthritis polyarthritis with injury of large and middle joints and has benign currency Injury of the nervous system with manifestation of hyperkinetic forms of rheumatic encephalitis with Chorea main developing of the pathological process in striapallidum region (muscle hypotonic, static’s and coordination disorders, vessels dystonia, psychoemotional changes) Pale-pink exanthema like thin line with strong Erythema marginatum external border, that doesn’t raise up over the skin level and disappear during its pressing Round, compact, poor moveable, painless forms with Rheumatic nodules sizes from millet corn to beans with localization on unbend join surface, vertebral prominent, occipital region Juvenile rheumatoid arthritis American College of Rheumatology: three types of onset: oligo (pauciarticular), polyarticular, & systemic in the first 6 months of onset

JIA proposed by the Pediatric Task force of the International League of Associations for Rheumatology ILAR – developed to achieve homogeneity within Juvenile Idiopathic Arthritis disease and categories. Arthritis of unknown etiology with duration of more than 6 weeks that develops in children under 16 years of age, upon exclusion of other joint pathology. Arthralgia pain in one or more joints. inflammation of joints in which a person's joints become Arthritis painful, swollen and stiff.

4.2. The theoretical questions to the studies: 1. Modern view on etiology of acute rheumatic fever. Risk factors of ARF development. 2. Pathogenesis, pathomorphology of acute rheumatic fever. 3. Clinical classification of acute rheumatic fever. 4. Diagnostic criteria of rheumatism Kisel-Johns. 5. Clinical picture of heart signs of ARF: endocarditis, myocarditis, pericarditis, and pancarditis. 6. Clinical picture of out heart signs of rheumatism: small chorea, polyarthritis, skin injuries. 7. Clinical picture in dependence of severity degree and activity of the process. 8. Additional methods of diagnostic of ARF (laboratory, instrumental, immunological, microbiological). 9. Differential diagnosis: nonrheumatic carditis, secondary cardiomyopathies, idiopathic prolapsed tricuspid valvule, other signs of dysplasia of conjunctive tissue syndrome, reactive arthritis, rheumatoid arthritis joints injury in case of other systemic diseases of conjunctive tissue. 10. Treatment principles of acute rheumatic fever in hospital, rest-house and polyclinic stages. Primary and secondary prevention. 11. Determination, risk factors etiology and pathogeny JRA for children. 12. In what essence of immunological theory Juvenile rheumatoid arthritis ? 13. Clinic. Classification JRA for children. 14. Principles of laboratory and functional diagnostics are JRA. 15. Reactive аrtropaty for children. Determination 16. Etiology and pathogeny. 17. Clinic of reactive аrtropaty. 18. Diagnosis and treatment JRA and reactive аrtropaty for children.

4.3. The practical works (tasks) which are done at the studies: 1. Work with test tasks. 2. Work of students in chambers near the bed of children of patients with ARF, Juvenile rheumatoid arthritis. 3. Interpretation of additional methods of inspection and differential diagnostics of diseases. 4. Clinical analysis of a typical case. 5. Solving of the situational tasks.

5. The contents of the topic:

Contents of topic is exhaustively expounded in the following sources: lecture, educational and scientific literature, information in the Internet network. Rheumatic fever (RF) is a systemic illness that may occur following group A beta hemolytic streptococcal (GABHS) pharyngitis in children. Rheumatic fever and its most serious complication, rheumatic heart disease (RHD), are believed to result from an autoimmune response; however, the exact pathogenesis remains unclear. The current incidence of rheumatic fever after GABHS infection is now thought to have decreased to less than 1%. Cardiac involvement is reported to occur in 30-70% of patients with their first attack of rheumatic fever and in 73-90% of patients when all attacks are counted. Rheumatic fever occurs in equal numbers in males and females. Females with rheumatic fever fare worse than males and have a slightly higher incidence of chorea. Rheumatic fever is principally a disease of childhood, with a median age of 10 years; However, GABHS pharyngitis is uncommon in children younger than 3 years, and acute rheumatic fever is extremely rare in these younger children in industrialized countries. Pathophysiology Rheumatic fever develops in children and adolescents following pharyngitis with GABHS (ie, Streptococcus pyogenes). The organisms attach to the epithelial cells of the upper respiratory tract and produce a battery of enzymes, which allows them to damage and invade human tissues. After an incubation period of 2-4 days, the invading organisms elicit an acute inflammatory response, with 3-5 days of sore throat, fever, malaise, headache, and elevated leukocyte count. In a small percent of patients, infection leads to rheumatic fever several weeks after the sore throat has resolved. Only infections of the pharynx have been shown to initiate or reactivate rheumatic fever. However, epidemiological associations in certain populations have led to speculation that group A Streptococcus impetigo could predispose to or cause rheumatic fever as well. Acute RHD often produces a pancarditis, characterized by endocarditis, myocarditis, and pericarditis. Endocarditis is manifested as mitral and aortic valve insufficiency. Severe scarring of the valves develops during a period of months to years after an episode of acute rheumatic fever, and recurrent episodes may cause progressive damage to the valves. The mitral valve is affected most commonly and severely (65-70% of patients); the aortic valve is affected second most commonly (25%). Clinical Acute rheumatic fever (RF) is a systemic disease. Thus, patients may present with a large variety of symptoms and complaints. • History of an antecedent sore throat 1-5 weeks prior to onset is present in 70% of older children and young adults. Only 20% of younger children can recall an antecedent sore throat. • Other symptoms on presentation may include fever, rash, headache, weight loss, epistaxis, fatigue, malaise, diaphoresis, and pallor. • Patients also may have chest pain with or abdominal pain and vomiting. • Finally, history may reveal symptoms more specific to rheumatic fever. o Migratory joint pain o Nodules under the skin o Increased irritability and shortened attention span with personality changes, such as pediatric autoimmune neuropsychiatric disorder associated with streptococcal infections (PANDAS) o Motor dysfunction o History of previous rheumatic fever • Patients with previous rheumatic fever are at a high risk of recurrence. o Highest risk of recurrence within 5 years of the initial episode o Greater risk of recurrence with younger age at the time of the initial episode o Generally, recurrent attacks similar to the initial attack (however, risk of carditis and severity of valve damage increase with each attack) Physical Revised in 1992, the modified Jones criteria provide guidelines for making the diagnosis of rheumatic fever.The Jones criteria require the presence of 2 major or 1 major and 2 minor criteria for the diagnosis of rheumatic fever. Having evidence of previous group A streptococci (GAS) pharyngitis is also necessary. These criteria are not absolute, and the diagnosis of rheumatic fever can be made in patients with only confirmed streptococcal pharyngitis and chorea. • Major diagnostic criteria o Carditis o Polyarthritis o Chorea o Subcutaneous nodules o Erythema marginatum • Minor diagnostic criteria o Fever o Arthralgia o Prolonged PR interval on electrocardiography o Elevated acute-phase reactants (APRs), which are erythrocyte sedimentation rate and C- reactive protein • Three notable exceptions to strict adherence to the Jones criteria o Chorea: It may occur late and be the only manifestation of rheumatic fever. o Indolent carditis: Patients presenting late to medical attention months after the onset of rheumatic fever may have insufficient support to fulfill the criteria. o Newly ill patients with a history of rheumatic fever, especially rheumatic heart disease (RHD), who have supporting evidence of a recent GAS infection and who manifest either a single major or several minor criteria: Distinguishing recurrent carditis from preexisting significant RHD may be impossible. Evidence of previous GAS pharyngitis (One of the following must be present): Positive throat culture or rapid streptococcal antigen test; Elevated or rising streptococcal antibody titer; Major clinical manifestations: Arthritis: Polyarthritis is the most common symptom and is frequently the earliest manifestation of acute rheumatic fever (70-75%). Characteristically, the arthritis begins in the large joints of the lower extremities (ie, knees, ankles) and migrates to other large joints in the lower or upper extremities (ie, elbows, wrists). Affected joints are painful, swollen, warm, erythematous, and limited in their range of motion. The pain is out of proportion to clinical findings. The arthritis reaches maximum severity in 12-24 hours and persists for 2-6 days (rarely more than 4 wk, but has been reported to persist 44 d) at each site and is migratory but not additive. The arthritis responds rapidly to aspirin, which decreases symptoms in affected joints and prevents further migration of the arthritis. Polyarthritis is more common and more severe in teenagers and young adults than in younger children. Patients suffering multiple attacks may exhibit destructive arthritis (Jaccoud arthritis). Carditis Pancarditis is the most serious complication and the second most common complication of rheumatic fever (50%). In advanced cases, patients may experience of dyspnea, mild-to-moderate chest discomfort, pleuritic chest pain, edema, cough, or orthopnea. Upon physical examination, carditis is most commonly revealed by a new murmur and tachycardia that is out of proportion to the fever. New or changing murmurs traditionally have been considered necessary for a diagnosis of rheumatic valvulitis. The murmurs of acute rheumatic fever are from valve regurgitation, and the murmurs of chronic rheumatic fever are from valve stenosis. Frequently examine patients in whom the diagnosis of acute rheumatic fever is made due to the progressive nature of the disease. Some cardiologists have proposed that evidence of new mitral regurgitation from Doppler echocardiography, even in the absence of accompanying auscultatory findings, may be sufficient for making the diagnosis of carditis, particularly if the echocardiography findings resolve along with other manifestations of rheumatic fever. This criterion for carditis is not uniformly accepted and remains specifically excluded in the 1992 revised Jones criteria because of insufficient data at the time of publication. Congestive heart failure (CHF) may develop secondary to severe valve insufficiency or myocarditis. Physical findings associated with heart failure include tachypnea, orthopnea, jugular venous distention, rales, hepatomegaly, a gallop rhythm, and peripheral swelling and edema. A pericardial friction rub indicates that pericarditis is present. Increased cardiac dullness to percussion, muffled heart sounds, and a paradoxical pulse are consistent with pericardial effusion and impending pericardial tamponade. Confirm this clinical emergency with ECG, and evacuate the effusion by pericardiocentesis if it is producing hemodynamic compromise. Chorea: In the absence of a family history of Huntington chorea or findings consistent with systemic lupus erythematosus, the diagnosis of acute rheumatic fever is almost certain. A long latency period exists between streptococcal pharyngitis (1-6 mo) and the onset of chorea, and a history of an antecedent sore throat frequently is not obtained. Patients with chorea often do not demonstrate other Jones criteria. Chorea is slightly more common in females than males. Chorea is also known as rheumatic chorea, Sydenham chorea, chorea minor, and St Vitus dance.Poststreptococcal movement disorders . Described poststreptococcal movement disorders have included pediatric autoimmune neuropsychiatric disorder associated with streptococcal infections (PANDAS) and Tourette syndrome. . Daily handwriting samples can be used as an indicator of progression or resolution of disease. Complete resolution of the symptoms typically occurs, with improvement in 1-2 weeks and full recovery in 2-3 months; however, incidents have been reported in which symptoms wax and wane for several years. . The PANDAS disorder appears to have a relapsing-remitting symptom complex characterized by obsessive-compulsive personality disorder. Patients with Sydenham chorea and obsessive- compulsive symptoms tend to show aggressive, contamination, and somatic obsessions and checking, cleaning, and repeating compulsions. Neurologic abnormalities include cognitive defects and motoric hyperactivity. The symptoms may also include emotional lability, separation anxiety, and oppositional behaviors, and they are prepubertal in onset. . Some have proposed that the streptococcal infection triggers the formation of antibodies that cross-react with the basal ganglia of genetically susceptible hosts in a manner similar to the proposed mechanism for Sydenham chorea and causes the symptom complex. o Erythema marginatum: This characteristic rash, also known as erythema annulare, occurs in 5-13% of patients with acute rheumatic fever. Erythema marginatum begins as 1-cm to 3-cm diameter, pink-to-red nonpruritic macules or papules located on the trunk and proximal limbs but never on the face. The lesions spread outward to form a serpiginous ring with erythematous raised margins and central clearing. The rash may fade and reappear within hours and is exacerbated by heat. Thus, if the lesions are not observed easily, they can be accentuated by the application of warm towels, a hot bath, or the use of tangential lighting. The rash occurs early in the course of the disease and remains long past the resolution of other symptoms. Erythema marginatum (shown in the image below) has also been reported in association with sepsis, drug reactions, and glomerulonephritis. Erythema marginatum, the characteristic rash of acute rheumatic fever. o Subcutaneous nodules: Subcutaneous nodules are now an infrequent manifestation of rheumatic fever. The frequency has declined during the past several years to 0-8% of patients with rheumatic fever. When present, the nodules appear over the extensor surfaces of the elbows, knees, ankles, knuckles, scalp, and spinous processes of the lumbar and thoracic vertebrae (attached to the tendon sheath). The nodules are firm, nontender, and free from attachments to the overlying skin, and they range from a few millimeters to 1-2 cm. The nodules number from 1 to dozens, with a mean of 3-4. Histologically, the nodules contain areas resembling the Aschoff bodies observed in the heart. Subcutaneous nodules generally occur several weeks into the disease and resolve within a month. They are strongly associated with severe rheumatic carditis, and in the absence of carditis, question the diagnosis of subcutaneous nodules. • Other clinical manifestations o Abdominal pain: Abdominal pain usually occurs at the onset of acute rheumatic fever, resembles other conditions with acute microvascular mesenteric inflammation, and may mimic acute appendicitis. o Arthralgias: Patients may report arthralgias upon presentation. In the history, determining if the patient has taken aspirin or nonsteroidal anti-inflammatory drugs (NSAIDs) is important because these may suppress the full manifestations of the disease. Arthralgia cannot be considered a minor manifestation if arthritis is present. o Epistaxis: Epistaxis may be associated with severe protracted rheumatic carditis. o Fever: Fevers greater than 39°C with no characteristic pattern are present initially in almost every patient with acute rheumatic fever. The fever may be low grade (38-38.5°C) in children with mild carditis or absent in patients with pure chorea. The fever decreases without antipyretic therapy in approximately 1 week, but low-grade fevers persist for 2-3 weeks. o Rheumatic pneumonia: Patients present with the same signs as an infectious pneumonia. Differentiate rheumatic pneumonia from respiratory distress related to CHF. Prevention of rheumatic fever in patients with group A beta hemolytic streptococci (GABHS) pharyngitis • Oral (PO) penicillin V remains the drug of choice for treatment of GABHS pharyngitis, but ampicillin and amoxicillin are equally effective. • When PO penicillin is not feasible or dependable, a single dose of intramuscular benzathine penicillin G, or benzathine/procaine penicillin combination is therapeutic. • For patients who are allergic to penicillin, administer erythromycin or a first-generation cephalosporin. Other options include clarithromycin for 10 days, azithromycin for 5 days, or a narrow- spectrum (first-generation) cephalosporin for 10 days. As many as 15% of penicillin-allergic patients are also allergic to cephalosporins. • Do not use tetracyclines and sulfonamides to treat GABHS pharyngitis. • For recurrent group A streptococci (GAS) pharyngitis, a second 10-day course of the same antibiotic may be repeated. Alternate drugs include narrow-spectrum cephalosporins, amoxicillin-clavulanate, dicloxacillin, erythromycin, or other macrolides. • Control measures for patients with GABHS pharyngitis are as follows: o Hospitalized patients: Place hospitalized patients with GABHS pharyngitis of pneumonia on droplet precautions, as well as standard precautions, until 24 hours after initiation of appropriate antibiotics. o Exposed persons: People in contact with patients having documented cases of streptococcal infection first should undergo appropriate laboratory testing if they have clinical evidence of GABHS infection and should undergo antibiotic therapy if infected. o School and childcare centers: Children with GABHS infection should not attend school or childcare centers for the first 24 hours after initiating antimicrobial therapy. • GABHS carriage is difficult to eradicate with conventional penicillin therapy. Thus, PO clindamycin (20 mg/kg/d PO in 3 divided doses for 10 d) is recommended. • In general, antimicrobial therapy is not indicated for pharyngeal carriers of GABHS. Exceptions include the following: o Outbreaks of rheumatic fever or poststreptococcal glomerulonephritis o Family history of rheumatic fever o During outbreaks of GAS pharyngitis in a closed community o When tonsillectomy is considered for chronic GABHS carriage o When multiple episodes of documented GABHS pharyngitis occur within a family despite appropriate therapy o Following GAS toxic shock syndrome or necrotizing fasciitis in a household contact Treatment for patients with rheumatic fever Therapy is directed towards eliminating the GABHS pharyngitis (if still present), suppressing inflammation from the autoimmune response, and providing supportive treatment of congestive heart failure (CHF). • Treat residual GABHS pharyngitis as outlined above, if still present. • Treatment of the acute inflammatory manifestations of acute rheumatic fever consists of salicylates and steroids. Aspirin in anti-inflammatory doses effectively reduces all manifestations of the disease except chorea, and the response typically is dramatic. o If rapid improvement is not observed after 24-36 hours of therapy, question the diagnosis of rheumatic fever. o Attempt to obtain aspirin blood levels from 20-25 mg/dL, but stable levels may be difficult to achieve during the inflammatory phase because of variable GI absorption of the drug. Maintain aspirin at anti- inflammatory doses until the signs and symptoms of acute rheumatic fever are resolved or residing (6-8 wk) and the acute phase reactants (APRs) have returned to normal. o Anti-inflammatory doses of aspirin may be associated with abnormal liver function tests and GI toxicity, and adjusting the aspirin dosage may be necessary. o When discontinuing therapy, withdraw aspirin gradually over weeks while monitoring the APRs for evidence of rebound. Chorea most frequently is self-limited but may be alleviated or partially controlled with phenobarbital or diazepam. • If moderate to severe carditis is present as indicated by cardiomegaly, third-degree heart block, or CHF, add PO prednisone to salicylate therapy. o Continue prednisone for 2-6 weeks depending on the severity of the carditis, and taper prednisone during the last week of therapy. o Discontinuing prednisone therapy after 2-4 weeks, while maintaining salicylates for an additional 2-4 weeks, can minimize adverse effects. • Include digoxin and diuretics, afterload reduction, supplemental oxygen, bed rest, and sodium and fluid restriction as additional treatment for patients with acute rheumatic fever and CHF. The diuretics most commonly used in conjunction with digoxin for children with CHF include furosemide and spironolactone. o Initiate digoxin only after checking electrolytes and correcting abnormalities in serum potassium. o The total loading dose is 20-30 mcg/kg PO every day, with 50% of the dose administered initially, followed by 25% of the dose 8 hours and 16 hours after the initial dose. Maintenance doses typically are 8-10 mcg/kg/d PO in 2 divided doses. For older children and adults, the total loading dose is 1.25-1.5 mg PO, and the maintenance dose is 0.25-0.5 mg PO every day. Therapeutic digoxin levels are present at trough levels of 1.5-2 ng/mL. • Afterload reduction (ie, using ACE inhibitor captopril) may be effective in improving cardiac output, particularly in the presence of mitral and . Start these agents judiciously. Use a small, initial test dose (some patients have an abnormally large response to these agents), and administer only after correcting hypovolemia. • When heart failure persists or worsens during the acute phase after aggressive medical therapy, surgery is indicated to decrease valve insufficiency. • Drug Category: • Because of the direct link between ARF and group A beta-streptococcal infection, the first step in treatment is the eradication of the organism. Antibiotic regimens used for prevention of recurrence are mentioned briefly under Further Outpatient Care.

Drug Name Penicillin G benzathine (Bicillin LA) Interferes with synthesis of cell wall mucopeptide during active multiplication, resulting in bactericidal activity against susceptible bacteria. Description Because of its prolonged blood level, several authors believe this to be the DOC. Others prefer daily injections. Adult Dose 2.4 million U IM once Infants and children <30 lb: 600,000 U IM once Pediatric Dose Children 30-60 lb: 900,000 to 1.2 million U IM once Drug Name Penicillin G procaine (Crysticillin, Wycillin) Long-acting parenteral penicillin (IM only) indicated in the treatment of moderately severe infections caused by penicillin G–sensitive microorganisms. Some prefer 10-d therapy. Description Administer by deep IM injection only into the upper outer quadrant of the buttock. In infants and small children, the midlateral aspect of the thigh may be the best site for administration. Adult Dose 2.4 million U IM once Infants and children <30 lb: 600,000 U IM Pediatric Dose Children 30-60 lb: 900,000 to 1.2 million U IM Drug Name Penicillin VK (Beepen-VK, Betapen-VK, Robicillin VK, Veetids) Inhibits the biosynthesis of the cell-wall mucopeptide and is effective during the stage of active multiplication. Inadequate concentrations may produce only Description bacteriostatic effects. Penicillin VK is the oral alternative for the treatment of rheumatic fever. Adult Dose 500 mg PO q6h for 10 d <12 years: 25-50 mg/kg/d PO divided tid/qid; not to exceed 3 g/d Pediatric Dose >12 years: Administer as in adults Drug Name Erythromycin (EES, E-Mycin, Ery-Tab, Erythrocin) DOC for patients allergic to penicillin; inhibits RNA-dependent protein synthesis, possibly by stimulating the dissociation of peptidyl tRNA from ribosomes, which Description inhibits bacterial growth. In children, age, weight, and severity of infection determine the proper dosage. When bid dosing is desired, one-half the daily dose may be administered q12h. For more severe infections, the dose may be doubled. Adult Dose 1 g/d PO divided bid for 10 d Pediatric Dose 30-50 mg/kg/d PO divided bid

• Drug Category: • These agents possess anti-inflammatory (ie, ) and salt-retaining (ie, ) properties. Glucocorticoids cause profound and varied metabolic effects. In addition, these agents modify the body's immune response to diverse stimuli. Drug Name Prednisone (Deltasone, Sterapred) Patients with carditis require prednisone instead of aspirin. The goal is to decrease myocardial inflammation. Some authors suggest that carditis without associated cardiomegaly or congestive heart failure be treated with aspirin instead of Description glucocorticoids. Glucocorticoids are useful in treatment of inflammatory and autoimmune disorders. Reversing increased capillary permeability and suppressing PMN activity may decrease inflammation. Adult Dose 60-80 mg/d PO Pediatric Dose 2 mg/kg/d PO

• Drug Category: Neuroleptic agents • These agents may help to control the chorea associated with ARF. Drug Name Haloperidol (Haldol) A dopamine receptor blocker useful in the treatment of irregular spasmodic Description movements of limbs or facial muscles. Adult Dose 0.5-2 mg PO bid/tid <3 years: Not established 3-12 years: 0.05 mg/kg/d or 0.25-0.5 mg/d bid/tid; increase by 0.25-0.5 mg q5-7d Pediatric Dose Maintenance dose: 0.05-0.15 mg/kg/d bid/tid; not to exceed 0.15 mg/kg/d >12 years: Administer as in adults

• Drug Category: Inotropic agents • Some believe that digoxin may be helpful in congestive heart failure. Drug Name Digoxin (Lanoxin) Cardiac with direct inotropic effects and indirect effects on the cardiovascular system. Effects on the myocardium involve a direct action on cardiac muscle that increases Description myocardial systolic contractions and indirect actions that result in increased carotid sinus nerve activity and enhanced sympathetic withdrawal for any given increase in mean arterial pressure. Adult Dose 0.125-0.375 mg PO qd Digitalizing dose: <2 years: Not established 2-5 years: 30-40 mcg/kg PO Pediatric Dose 5-10 years: 20-35 mcg/kg PO >10 years: 10-15 mcg/kg PO Maintenance dose: 25-35% of PO loading dose

• Drug Category: Anti-inflammatory agents • Reduce the inflammation associated with the disease process. Joints and heart are the targets of inflammation, but carditis is treated with glucocorticoids as noted above. Drug Name Aspirin (Ascriptin, Bayer Buffered Aspirin, Ecotrin) Treats mild to moderate pain. Inhibits prostaglandin synthesis, which prevents Description formation of -aggregating thromboxane A2. Adult Dose 6-8 g/d PO for 2 mo or until ESR has returned to normal Pediatric Dose 80-100 mg/kg/d PO for 2 mo or until ESR has returned to normal Drug Name Naproxen (Anaprox, Naprelan, Naprosyn) For relief of mild to moderate pain; inhibits inflammatory reactions and pain by decreasing activity of cyclooxygenase, which is responsible for prostaglandin Description synthesis. NSAIDs decrease intraglomerular pressure and decrease proteinuria. Adult Dose 250-500 mg PO bid; may increase to 1.5 g/d for limited periods <2 years: Not established Pediatric Dose >2 years: 2.5 mg/kg/dose PO; not to exceed 10 mg/kg/d Deterrence/Prevention • Literature, much of it now dated, reports that ARF can effectively be prevented if appropriate antibiotics are given within 9 days of symptom onset. Most authorities believe this to be a valid conclusion. • Differences exist among nations in terms of diagnosing and treating GABHS pharyngitis. Most North American, French, and Finnish guidelines consider diagnosis of streptococcal infection essential (with either rapid antigen detection or with formal culture) and advise antibiotic therapy when streptococci is detected. Several European guidelines consider streptococcal infection a self- limited disease and do not recommend antibiotics. The North American guidelines refer primarily to North American studies. European guidelines did not reference North American studies as frequently. Complications: • Carditis • Mitral stenosis • Mitral regurgitation • Aortic stenosis • Congestive heart failure (CHF) Prognosis. Sequelae are limited to the heart and are dependent upon the severity of the carditis during the acute attack.

Treatment for patients following rheumatic heart disease (RHD) Preventive and prophylactic therapy is indicated after rheumatic fever and RHD to prevent further damage to valves. • Primary prophylaxis (initial course of antibiotics administered to eradicate the streptococcal infection) also serves as the first course of secondary prophylaxis (prevention of recurrent rheumatic fever and RHD). • An injection of 0.6-1.2 million units of benzathine penicillin G intramuscularly every 4 weeks is the recommended regimen for secondary prophylaxis for most US patients. Administer the same dosage every 3 weeks in areas where rheumatic fever is endemic, in patients with residual carditis, and in high-risk patients. o Although PO penicillin prophylaxis is also effective, data from the World Health Organization indicate that the recurrence risk of GABHS pharyngitis is lower when penicillin is administered parentally. o Ideally, continue prophylaxis indefinitely, because recurrent GABHS infection and rheumatic fever can occur at any age; however, the American Heart Association currently recommends that patients with rheumatic fever without carditis receive prophylactic antibiotics for 5 years or until aged 21 years, whichever is longer. Patients with rheumatic fever with carditis but no valve disease should receive prophylactic antibiotics for 10 years or well into adulthood, whichever is longer. Finally, patients with rheumatic fever with carditis and valve disease should receive antibiotics at least 10 years or until aged 40 years. o Patients with RHD and valve damage require a single dose of antibiotics 1 hour before surgical and dental procedures to help prevent bacterial endocarditis. Patients who had rheumatic fever without valve damage do not need endocarditis prophylaxis. Do not use penicillin, ampicillin, or amoxicillin for endocarditis prophylaxis in patients already receiving penicillin for secondary rheumatic fever prophylaxis (relative resistance of PO streptococci to penicillin and aminopenicillins). Alternate drugs recommended by the American Heart Association for these patients include PO clindamycin (20 mg/kg in children, 600 mg in adults) and PO azithromycin or clarithromycin (15 mg/kg in children, 500 mg in adults). • Juvenile idiopathic arthritis (JIA) is a collection of chronic idiopathic autoimmune non-infectious arthritides. By definition, disease onset is prior to 16 years of age and includes joint inflammation that is present for 6 weeks or more. JIA affects approximately 1 in 1,000 children and approximately 50% of children have oligoarticular disease (involves 4 or fewer joints), 40% have polyarticular (involves 5 or more joints), and ~10% have systemic symptoms along with arthritis (i.e., systemic arthritis). • The aetiology and pathogenesis of JIA remains unclear. Abnormal immunoregulation and cytokine production, genetic predisposition of immune response and latent viral infection may play a role. JIA is characterised by many of the same histologic abnormalities that have been identified in rheumatoid arthritis (RA). The production of large amounts of cytokines (interleukin-1ß and-6, TNF-α etc) which in some subtypes of JIA even exceeds cytokine excess in RA, results in hyperplastic synovial membrane and in conjunction with osteoclastic cell activation leads to degradation of adjacent cartilage and bone. Disturbance and retardation of growth in various aspects are characteristic features of JIA. • Classification • Juvenile idiopathic arthritis is not a single disease; the term applies to a number of chronic arthritides that occur in children and share certain features. The current classification system, from the International League of Associations for Rheumatology, defines categories of disease based on clinical and laboratory findings. Some of the categories are subdivided into different forms. Categories include the following: • 1. Systemic arthritis • 2. Polyarthritis, RF-negative • 3. Polyarthritis, RF-positive • 4. Oligoarthritis (persistent, extended) • 5. Psoriatic arthritis • 6. Enthesitis-related arthritis • 7. Other Arthritis

Criteria for the Classification Age at onset: <16 yr Arthritis (swelling or effusion, or the presence of 2 or more of the following signs: limitation of range of motion, tenderness or pain on motion, increased heat) in ≥1 joints Duration of disease: ≥ 6 wk Onset type defined by type of articular involvement in the 1st 6 mo after onset: Polyarthritis: ≥5 inflamed joints Oligoarthritis: ≤4 inflamed joints Systemic disease: arthritis with a characteristic intermittent fever Exclusion of other forms of juvenile arthritis Characteristics of the ACR, EULAR, and ILAR Classifications of Chronic Arthritis in Children EUROPEAN LEAGUE INTERNATIONAL LEAGUE OF AMERICAN COLLEGE OF CHARACTERISTIC AGAINST RHEUMATISM ASSOCIATIONS FOR RHEUMATOLOGY (ACR) (ELAR) RHEUMATOLOGY (ILAR) Onset types 3 6 6 Course subtypes 9 None 1 Age at onset of <16 yr <16 yr <16 yr arthritis Duration of arthritis ≥6 wk ≥3 mo ≥6 wk Includes juvenile No Yes Yes ankylosing spondylitis EUROPEAN LEAGUE INTERNATIONAL LEAGUE OF AMERICAN COLLEGE OF CHARACTERISTIC AGAINST RHEUMATISM ASSOCIATIONS FOR RHEUMATOLOGY (ACR) (ELAR) RHEUMATOLOGY (ILAR) Includes juvenile No Yes Yes psoriatic arthritis Includes inflammatory No Yes Yes bowel disease Exclusion of other Yes Yes Yes diseases

• Oligoarticular JIA is the most common form and usually affects young girls. It is characterized by involvement of ≤ 4 joints during the first 6 mo of disease. Oligoarticular JIA is further divided into 2 types: persistent (always ≤ 4 joints involved) and extended (≥ 5 joints involved after the first 6 mo of disease). • Polyarticular JIA is the second most common form. It affects ≥ 5 joints at onset and is divided into 2 types: RF negative and RF positive. Typically, young girls are RF negative and have a better prognosis. The RF-positive type typically occurs in adolescent girls and is often similar to adult RA. In both types, arthritis can be symmetric and frequently involves the small joints. • Enthesitis-related arthritis involves arthritis and enthesitis (painful inflammation at the insertion of tendons and ligaments). It is more common among older boys who may subsequently develop classic features of one of the spondyloarthritides such as ankylosing spondylitis or reactive arthritis. The arthritis tends to be in the lower extremities and asymmetric. The HLA-B27 allele is more common in this form of JIA. • Psoriatic JIA has a bimodal age distribution. One peak occurs in young girls, and the other peak occurs in older males and females (who are equally affected). It is associated with psoriasis, dactylitis (swollen digits), nail pits, or a family history of psoriasis in a 1st-degree relative. Arthritis is frequently oligoarticular. • Undifferentiated JIA is diagnosed when patients do not meet criteria for any one category or meet criteria for more than one. • Systemic JIA (Still disease) involves fever and systemic manifestations. • Symptoms and Signs • Manifestations involve the joints and sometimes the eyes and/or skin; systemic juvenile idiopathic arthritis may affect multiple organs. The cardinal clinical features are persistent swelling and pain of the joints. Morning stiffness may be present, and typically improves throughout the day with joint use. Linear growth delay can occur in children with JIA, and untreated arthritis can lead to severe joint deformities and disability. Uveitis is the most common extra-articular manifestation and can lead to ocular complications and permanent vision loss. Regular screening by ophthalmology for early detection and timely treatment is crucial. • Children typically have joint stiffness, swelling, effusion, pain, and tenderness, but some children have no pain. Joint manifestations may be symmetric or asymmetric, and involve large or small joints. Enthesitis typically causes tenderness of the iliac crest and spine, greater trochanter of the femur, patella, tibial tuberosity, or Achilles and plantar fascia insertions. • Sometimes, JIA interferes with growth and development. Micrognathia (receded chin) due to early closure of mandibular epiphyses or limb length inequality (usually the affected limb is longer) may occur. • The most common ocular abnormality is iridocyclitis (inflammation of the anterior chamber and anterior vitreous) that is typically asymptomatic but sometimes causes blurring of vision and miosis. Rarely, in enthesitis-related arthritis, there is conjunctival injection, pain, and photophobia. Iridocyclitis can result in scarring (synechia), cataracts, glaucoma, or band keratopathy. Iridocyclitis is most common in oligoarticular JIA, developing in nearly 20% of patients, especially if patients are positive for antinuclear antibodies (ANA). It may occur in the other forms but is rare in polyarticular RF-positive JIA and systemic JIA. • Skin abnormalities are present mainly in psoriatic JIA, in which psoriatic skin lesions, dactylitis, and/or nail pits may be present, and in systemic JIA, in which a typical transient rash often appears with fever. Rash in systemic JIA may be diffuse and migratory, with urticarial or macular lesions with central clearing. • Systemic abnormalities in systemic JIA include high fever, rash, , generalized adenopathy (especially of the axillary nodes), and serositis with pericarditis or pleuritis. These symptoms may precede the development of arthritis. Fever occurs daily (quotidian) and is often highest in the afternoon or evening and may recur for weeks. • Diagnosis • a) Risk factors:female sex, HLA polymorphism, age under 6 years, family history of autoimmunity; • b) Clinical criteria: presence of risk factors, more than 6 weeks' duration, joint pain, joint swelling. Other diagnostic factors: morning stiffness, limp, limited movement, rash. • c) Rheumatoid factor (RF), antinuclear antibodies (ANA), anticyclic citrullinated peptide antibody (anti- CCP), and HLA-B27 tests, C-reactive protein (CRP) • Juvenile idiopathic arthritis should be suspected in children with symptoms of arthritis, signs of iridocyclitis, generalized adenopathy, splenomegaly, or unexplained rash or prolonged fever, especially if quotidian. Diagnosis of JIA is primarily clinical. Patients with JIA should be tested for RF, anti-CCP antibodies, ANA, and HLA-B27 because these tests may be helpful in distinguishing between forms. In systemic JIA, RF and ANA are usually absent. In oligoarticular JIA, ANA are present in up to 75% of patients and RF is usually absent. In polyarticular JIA, RF usually is negative, but in some patients, mostly adolescent girls, it can be positive. HLA-B27 is present more commonly in enthesitis-related arthritis.To diagnose iridocyclitis, a slit-lamp examination should be done even in the absence of ocular symptoms. A recently diagnosed patient with oligoarticular or polyarticular JIA should have an eye examination every 3 month if ANA test results are positive and every 6 mo if ANA test results are negative. • • Prognosis • Remissions occur in 50 to 70% of treated patients. Patients with RF-positive polyarticular JIA have a less favorable prognosis. • Treatment • Drugs that slow disease progression (particularly methotrexate, etanercept, and anakinra) • Intra-articular corticosteroid injections • NSAIDs • Similar to the therapy of patients with adult rheumatoid arthritis, disease-modifying antirheumatic drugs (DMARDs), particularly methotrexate and the biologic agents (eg, etanercept, anakinra), have dramatically changed the therapeutic approach. • First-line therapy includes symptom-modifying (or symptom-relieving) antirheumatic drugs (SMARDs), which are mainly nonsteroidal antiinflammatory drugs (NSAIDs). NSAIDs are used at the beginning of treatment, in addition to disease-modifying antirheumatic drugs (DMARDs), or alone when disease flares are intermittent and only mild to moderate. Systemic glucocorticoids (prednisone, methylprednisolone) are used to control systemic inflammation (e.g. uncontrollable fever and systemic “toxicity”, severe anaemia, myocarditis) in systemic-onset JIA, or in severe polyarticular JIA as an adjunct at the start of treatment with DMARDs. Intra-articular injection of long-acting glucocorticoids such as triamcinolone hexacetonide directly into inflamed joints has emerged as a major advance in the treatment of children with various types of arthritis. • Symptoms of juvenile idiopathic arthritis may be reduced with NSAIDs but they do not alter long-term joint disease or prevent complications. NSAIDs are most useful for enthesitis. Naproxen 5 to 10 mg/kg po bid, ibuprofen 5 to 10 mg/kg po qid, and indomethacin 0.5 to 1.0 mg/kg po tid are among the most useful. • Except for severe systemic disease, systemic corticosteroids can usually be avoided. When necessary, the lowest possible dose is used (eg, range for oral prednisone, 0.0125 to 0.5 mg/kg qid, or the same daily dose given once or twice daily). Growth retardation, osteoporosis, and osteonecrosis are the major hazards of prolonged corticosteroid use in children. Intra-articular corticosteroid injections can be given. The dosage for children is adjusted based on weight. A few children may need to be sedated for intra- articular injection, especially if multiple joints require injection. • Methotrexate is useful for oligoarticular, psoriatic, and polyarticular forms of JIA. Adverse effects are monitored as in adults. Bone marrow depression and hepatic toxicity are monitored with CBC, AST, ALT, and albumin. Occasionally, sulfasalazine is used, especially in cases of suspected spondyloarthropathy. • TNF-alpha blockade is used if methotrexate is not effective. The introduction of therapy with TNF receptor: fusion protein (etanercept), appears to have a clinically relevant impact on the outcome of patients with active polyarticular disease (which includes several categories of onset) and who were unresponsive to methotrexate. Treatment with other medicinal products such as different types of TNF (tumour necrosis factor) modulators (infliximab, adalimumab), IL-1 ra (Interleukin-1 receptor antagonist), anti-IL-6 receptor (anti-interleukin 6 receptor) and CTLA4ag (cytotoxic T-lymphocyte antigen) are currently under clinical investigation in trials suitable to fulfil regulatory requirements. • Etanercept is used most commonly at doses of 0.4 mg/kg sc (up to a maximum of 25 mg) twice/wk or 0.8 mg/kg sc (up to 50 mg) once/wk. Adalimumab and infliximab are different TNF-alpha antagonists that have been shown to be effective. IL-1 blockade with anakinra or canakinumab is particularly effective for systemic JIA. Tocilizumab is an IL-6 receptor antagonist that is indicated for the treatment of systemic JIA. • Physical therapy, exercises, splints, and other supportive measures may help prevent flexion contractures. Adaptive devices can improve function and minimize unnecessary stresses on inflamed joints. Iridocyclitis is treated with ophthalmic corticosteroid drops and mydriatics and may require systemic methotrexate and anti-TNF therapy. • Systemic JIA with active systemic features and varying degrees of synovitis. The TFP was asked to consider the treatments among patients with an MD global of 5≤or ≥5 on a 10-point numerical rating scale and by AJC (0 joints, 1–4 joints, or >4 joints). The TFP voting panel was informed that these patients could be assumed to be receiving concurrent systemic GC therapy and that they should rate the appropriateness of initiating the medication under consideration either with or without concurrent initiation or increase of GC therapy (whichever approach was considered more appropriate by the voter). The recommendations in this section are for patients with active systemic features. If the systemic features (but not the arthritis) respond to therapy, then subsequent treatment decisions should be based upon recommendations in the next section, “Systemic JIA without active systemic features and with varying degrees of active synovitis.”

6. The materials for self-control A. Sample case report Tasks. №1. Girl of 12 years was hospitalized to the pediatric department with complaints about increasing of temperature to 39C, pain, swelling of right knee. Disease started 3 weeks ago after . Girl often has infection diseases of upper airways and decompensate tonsillitis. Mother of the girl has tricuspid heart defect. Objective: St. lokalis – all 5 signs of inflammation of right knee are present. Heart rate 100 beats/min. BP 110/65 mm Hg. Heart borders: the left is moved on 1,5 cm out from l. medioclavicularis sin. Heart sounds are weakened, systolic murmur on the apex. ECG: interval P Q is prolonged. Tasks: 1. Put a diagnosis. 2. Work out a plan of investigation. 3. Work out a plan of treatment. 4. Write a prescription for Benzylpenicillini-natrii.

№2. Girl is 8 years of age. She was hospitalized in pediatric department with involuntarily movements of face muscles, upper extremities, changes of the hand writing. She had pharyngitis before 4 weeks of the appearance of indicated symptoms. Objective: body temperature 37.4 C, general condition is moderate. The girl is anxiety, thick. Left border of the heart is moved to the 1 cm out of left l. mediaclavicularis. Heart sounds are weakened, systolic murmur on the apex. ESR 25mm/h. Tasks: 1. Put a diagnosis. 2. Work out a plan of investigation. 3. Work out a plan of treatment. 4. Write a prescription for Phenobarbitalum (tab.)

№3. Boy of 14 years old was hospitalized to the clinic with complaints about vomiting, quick thickness, increasing of body temperature to 38,5 C. He is ill during 1 month. Disease started with abdominal and joint pain. He was treated at the hospital, after the treatment condition became better, but thickness and periodically subfebrile temperature were observed. During last week condition became worse, vomiting, abdominal pain and pain in heart region appeared. Objective: general condition of the boy is severe, the skin is pale, and foot swells. Left heart border on 2 cm out from l. mediaclavicular left. Heart sounds are weakened, systolic murmur on the apex, irradiates. Heart rate 120 beats/min. Liver + 4cm. Blood test: CRP ++++, CIA 0,4 units, seromucoid 0,45 units. T ASLO 1:1250. Tasks: 1. Put a diagnosis. 2. Work out a plan of investigation. 3. Work out a plan of treatment. 4. Write a prescription for Prednisolon.

№4. Boy of 9 years of age during the training falled down cause of thickness in left leg and hand. During the last month he noticed appearance of thickness, inconvenient during walking, periodical headache. A boy was sent to the hospital with diagnose: hemiplegia of unknown etiology. During the hospitalization condition is satisfactory. The skin is pale. Pharyngeal tonsils are hypertrophic. Hands are wet. There is severe muscle hypotonia in left extremities. During representation of finger-nasal test – disorder of movement coordination in left hand was found. Movements are quick, uncoordinated. Heart borders are spread to the left on 1 cm. Heart sounds are weakened, systolic murmur on the apex. ECG: sinus arrhythmia, AV block II d. ESR 15mm/h. Tasks: 1. Put a diagnosis. 2. Work out a plan of investigation. 3. Work out a plan of treatment. 4. Write a prescription for Benzathini benzylpenicillini (Retarpen).

№5. Boy of 12 years of age has complaints about increasing of temperature to 38,5 C, thickness, pain and losing of function in knee joints. He had viral infection month before but he didn’t receive treatment. In anamnesis he has often anginas. During examination: skin is pale, palmer capillaritis, angular exanthema on legs, joints of normal structure, but active movements are difficult because of pain. Heart sounds are weakened, systolic murmur on the apex. Heart rate 110/min. BP 95/60 m Hg. Liver +4 cm. Tasks: 1. Put a diagnosis. 2. Work out a plan of investigation. 3. Work out a plan of treatment. 4. Write a prescription for Nimesulidum.

№6. Child is four years. It is ill during 3 weeks. A disease was begun with getting up of temperature to 39 C. Na extent of days a temperature changed on 2-2,5 degree, a decline was accompanied drivings sweats. The rose rash of differentform and localization appeared periodically. Were ill and swelled up joints (genicular, talocrural, radiocarpal), a child did not stand on feet, could not take objects. Pain proceeded 1-2 days. Objectively: temperature 39 C, liver +2 see, spleen +1 see, all of groups of lymphonoduss are palpated. Talocrural joints were swollen, are painful, contours them smoothed out. Blood test: Hb of 112 , Er. 3,9x10/12, leucocytes 12,9x109, SSE 54 mm/hour.

A. Preliminary diagnosis? B. To make the supervision plan С. To make the treatment plan D. Work out a plan of inspection

№7. For a girl 8 years sudden in the morning there was pain in a right knee-joint, it was not able to get up from a bed, at a review there is the expressed slight swelling of the noted joint (circumference of right knee- joint on 2 see anymore left), local, hyperthermia, hyperemia. A temperature for a child is normal. Active and passive motions are very sickly. There was first-aid, a child is delivered to the hospital. Sciagraphy of right knee-joint is conducted, signs of defeat of bone fabric, it is not discovered. A. Preliminary diagnosis? B. To make the supervision plan С. To make the treatment plan D. Work out a plan of inspection

№8. Boy 13 years grumbles about pain and edema of talocrural joints, urodynia.There is hyperemia of conjunctiva of eyes at examination. Your diagnosis? A. Preliminary diagnosis? B. To make the supervision plan С. To make the treatment plan D. Work out a plan of inspection

№9. For a girl 5 years, the slight swelling and gonalgia appeared after intensifying of chronic tonsillitis. Infectiously-allergic arthritis was diagnosed. However through 5 months the oedematousness and pain appeared in talocrural, and then in an elbow joints. During clinical examination an iridocyclitis is exposed. A. Preliminary diagnosis? B. To make the supervision plan С. To make the treatment plan D. Work out a plan of inspection

№10. The mother of girl 12 years caused a district paediatrician and grumbles about that for a child pain and slight swelling of talocrural joint is marked. A doctor appointed compress to the staggered joint. In 2 weeks a pathological process de velops in other symmetric joint. Except for pains and limitation of motions in a joint, child of disturbs febricula, weakness, mass of body, subfebrilitet, goes down, SSE grows to 20-25 mm/hour. From anamnesis: before appearance of the first signs of disease, a child had a flu. At examination of – state child heavy. It can not show a leg, there is the expressed slight swelling of joints, local gipertermiya, hyperemia. Temperature of body 37,2 C. Active and passive motions very are limited. A. Preliminary diagnosis? B. To make the supervision plan С. To make the treatment plan D. Work out a plan of inspection

№11. Child is four years. It is ill during 3 weeks. A disease was begun with getting up of temperature to 39 C. Na extent of days a temperature changed on 2-2,5 degree, a decline was accompanied drivings sweats. The rose rash of differentform and localization appeared periodically. Were ill and swelled up joints (genicular, talocrural, radiocarpal), a child did not stand on feet, could not take objects. Pain proceeded 1-2 days. Objectively: temperature 39 C, liver +2, spleen +1, all of groups of lymphonoduss are palpated. Talocrural joints were swollen, are painful, contours them smoothed out. Blood test: Hb of 112 , Er. 3,9x10/12, leucocytes 12,9x109, ЕSR 54 mm/hour. 1. Make the preliminary diagnosis. 2. Make the plan of examination. 3. Make the plan of patient’s treatment. 4. Prescribe the child Diclofenаcum natrii

№12. For a girl 8 years sudden in the morning there was pain in a right knee-joint, it was not able to get up from a bed, at a review there is the expressed slight swelling of the noted joint (circumference of right knee- joint on 2 see anymore left), local, hyperthermia, hyperemia. A temperature for a child is normal. Active and passive motions are very sickly. There was first-aid, a child is delivered to the hospital. Sciagraphy of right knee-joint is conducted, signs of defeat of bone fabric, it is not discovered. 1. Make the preliminary diagnosis. 2. Make the plan of examination. 3. Make the plan of patient’s treatment. 4. Prescribe the child Meloxicamі.

№13. Boy 13 years grumbles about pain and edema of talocrural joints, urodynia.There is hyperemia of conjunctiva of eyes at examination. Your diagnosis? 1. Make the preliminary diagnosis. 2. Make the plan of examination. 3. Make the plan of patient’s treatment. 4. Prescribe the child Celecoxib-norton

№14. For a girl 5 years, the slight swelling and gonalgia appeared after intensifying of chronic tonsillitis. Infectiously-allergic arthritis was diagnosed. However through 5 months the oedematousness and pain appeared in talocrural, and then in an elbow joints. During clinical examination an iridocyclitis is exposed. 1. Make the preliminary diagnosis. 2. Make the plan of examination. 3. Make the plan of patient’s treatment. 4. Prescribe the child Metotrexat

№15. The mother of girl 12 years caused a district paediatrician and grumbles about that for a child pain and slight swelling of talocrural joint is marked. A doctor appointed compress to the staggered joint. In 2 weeks a pathological process de velops in other symmetric joint. Except for pains and limitation of motions in a joint, child of disturbs febricula, weakness, mass of body, subfebrilitet, goes down, ESR grows to 20-25 mm/hour. From anamnesis: before appearance of the first signs of disease, a child had a flu. At examination of – state child heavy. It can not show a leg, there is the expressed slight swelling of joints, local gipertermiya, hyperemia. Temperature of body 37,2 C. Active and passive motions very are limited. 1. Make the preliminary diagnosis. 2. Make the plan of examination. 3. Make the plan of patient’s treatment. 4. Prescribe the child Prednisolon

B. The tests for self-control 1. A 10-year-old boy with rheumatic fever was treated in cardiologic department. The disease occurred for the first time. He was discharged in satisfactory state. What preparation is the most advisable to prevent secondary rheumatic fever? A. Bicillin-5 B. Bicillin-1 C. Erythromicin D. Ampicillin E. Oxacillin

2. Rheumatic myocarditis was diagnosed in a 5-year-old child. ECG showed prolongation of PQ interval to 0.22”, inversion of T wave in chest leads. What function of the myocardium is disturbed according to ECG findings? A. Conduction B. Excitability C. Automatism D. Contractive activity E. All listed 3. A 10-year-old girl took a course of treatment at the in-patient department and sanatorium after primary attack of rheumatic fever associated with polyarthritis, skin erythema against the background of chorea. Blood analysis showed increased content of proteins of acute phase and high titre of antistreptococci antibodies. At present there are no signs of active rheumatic process. What optimal regime of bicillin prophylaxis will you choose? A. Bicillin-5 once in 3-4 weeks B. Bicillin-5 once a week C. Bicillin-5 in combination with prednisolone D. Bicillin-5 in combination with aspirin E. Bicillin prophylaxis only during intercurrent infection

4. Rheumocarditis was diagnosed in a 12-year-old boy 6 weeks after he had had tonsillitis. Activity of disease process corresponds to grade II. Antirheumatic therapy administered allowed to control laboratory signs of its activity by the end of the 3rd month of treatment when mitral valve insufficiency was diagnosed. Define the character of rheumatism course: A.Subacute B. Acute C. Prolonged D. Continuously recurrent E. Latent 5. An 8-year-old boy experienced “shifting” pains in the joints, their swelling and limited movements, fever 2 weeks after he had had tonsillitis. Diagnosis: rheumatism, activity of grade III. Primary rheumocarditis, polyarthritis, acute course. Circulatory insufficiency of grade II. Which of the following drugs should be administered? A. Prednisolone B. Orthofen C. Chloroquine D. Promethazine E. Erythromicine 6. A 10-year-old boy (the 3rd day of illness) complains of abdominal pain, pain and limited movements in his left knee and right elbow. He has had tonsillitis two weeks ago. In the first day of illness he had fever 38.5o C, affection of shin joints. The examination showed extension of borders of cardiac dullness by 2 cm, tachycardia 120 beats per minute, weakened 1st sound, gallop, “soft” systolic murmur at the heart apex. What diagnosis does the described clinical picture correspond to? A. Rheumatism B. Systemic lupus erythematosus C. Juvenile rheumatoid arthritis D. Reiter’s disease E. Reactive arthropathy 7. A 7-year-old girl complains of the fever 38o C, pain in the ankles and knees. She has had tonsillitis 3 weeks ago. The diagnosis of rheumatism was made. What is the etiological factor of this disease? A. Streptococcus B. Staphylococcus C. Clebciela D. Gonococcus E. Yersinia 8. A 9 year-old-boy had scarlet fever 2 weeks ago. He complains of general weakness, increased body temperature to 38oC, heart pain, dyspnea and skin paleness for 3 days. Auscultation revealed weakened heart sounds, systolic murmur above the apex, bifurcation of the second heart sound. ECG showed: prolongation of QP and PQ interval (0.22 c), decrease of T wave amplitude, solitary extrasystole. What disease will the district pediatrician suspect? A. Rheumatic carditis B. Cardiomyopathy C. Neurocirculatory dystonia D. Pneumonia E. Congenital heart disease 9. An 11 year-old girl had gradual development of the disease (she has the history of frequent tonsillitis). Irritability, inattention and hyperkinesia appeared. The examination revealed disturbed coordination of movements, muscle hypotonia, hyperrephlexia. What disease is characterized by such changes in central nervous system functioning? A. Rheumatism B. Systemic lupus erythematosus C. Systemic scleroderma D. Nodular periarteritis E. Vegetovascular dysfunction 10. The following skin manifestations occur in rheumatism: A. Annular erythema B. Nodular erythema C. Petechias D. Ecchimoses E. Morbilliform rash 11. An 8 year old child has low-grade fever, arthritis, colicky abdominal pain and a purpuric rash llocalized on the lower extremities. laboratory studies reveal a guaiac-positive stool, urinalysis with red blood cell (RBC) casts and mild proteinuria, and a normal platelet count. The most likely diagnosis is: A Henoch-Schonlein's vasculitis B Systemic lupus erythematosus (SLE) C Rocky Mountain spotted fever D Idiopathic thrombocytopenic purpura E JRA 12. A 7 y.o. child had elevation of temperature tol 40° in anamnesis. For the last 3 months he presents fusiform swelling of fingers, ankle joints and knee joint, pain in the upper part of the sternum and cervical part of the spinal column. What is the most probable diagnosis? A Juvenile rheumatic arthritis B Rheumatism C Toxic synovitis D Septic arthritis E Osteoarthrits 13. A 1,5-year-old child fell ill acutely with high temperature 38oC, headache, fatigue. The temperature declined on the fifth day, muscular pain in the right leg occured in the morning, there were no movements and tendon reflexes, sensitivity was reserved. What is the initial diagnosis? A. Hip joint arthritis B. Viral encephilitis C. Polyartropathy D. Osteomyelitis E. Polyomyelitis 14. A 17-year-old male patient has been suffering from pain in the sacrum and coxofemoral joints, painfulness and stiffness in the lumbar spine for a year. ESR- 56 mm/h. Roentgenography revealed symptoms of bilateral sacroileitis. The patient is the carrier of HLA B27 antigen. What is the most likely diagnosis? A. Spondylosis B. Ankylosing spondylitis C. Reiter's disease D. Rheumatoid arthritis E. Coxarthrosis 15. A 15-year-old patient complains about pain and morning stiffness of hand joints and temporomandibular joints that lasts over 30 minutes. She has had these symptoms for 2 years. Objectively: edema of proximal interphalangeal digital joints and limited motions of joints. What examination should be administered? A. Roentgenography of hands B. Rose-Waaler reaction C. Immunogram D. Proteinogram E. Complete blood count 16. A 17-year-old patient has been admitted to a hospital for pain in the left sternoclavicular and knee joints, lumbar area. The disease has an acute character and is accompanied by fever up to 38oC. Objectively: the left sternoclavicular and knee joints are swollen and painful. In blood: WBCs - 9,5x109/l, ESR - 40 mm/h, CRP - 1,5 millimole/l, fibrinogen - 4,8 g/l, uric acid - 0,28 millimole/l. Examination of the urethra scrapings reveals chlamydia. What is the most likely diagnosis? A. Reiter's syndrome B. Rheumatic arthritis C. Gout D. Rheumatoid arthritis E. Bechterew's disease 17. A 14-year-old female patient has a history of JRA. She complains about acute pain in her left eye, especially at night, vision impairment, photophobia, lacrimation. The patient cannot suggest any reasons for the disease. Objectively: weak pericorneal injection, flattening of iris relief, iris discoloration. What is the most likely diagnosis? A. Iridocyclitis B. Iritis C. Keratitis D. Choroiditis E. Acute attack of glaucoma 18. The boy 4 years within 3 months of knee swelling occurs, early stiffness, increased body temperature. In the synovial fluid revealed a significant amount of mucin and phagocyte. What is the most likely diagnosis? A. JRA B. HRA C. Reactive arthritis D. Rheumatic arthritis E. Post traumatic sinoviit left knee

7. Literature: Basic 1. Nelson Textbook of Pediatrica 18th edition. Richard E., Md Behrman, Robert M., Md Kllegman, Hal B., Md Jenaon. W.B.Saunders, 2007. 2. Behrman Richard E., Robert M. Kliegman, Hal B. Jenson, eds. Nelson Texbook of Paediatrics. Philadelfia: WB Saunders, 18th edition - 2012. – 994 р. – Р.638. 3. Buryak V.N., Makhmutov R.F., Poshehonova J.V. Children`s diseases. – Donetsk: Nord-Press, 2010. – 328 p. 4. Pediatry. Guidance aid / Ed. by O. Tiazhka – Kyiv: Medicine, 2007. 5. Shlopov VG Fundamentals pathological human anatomy. -Kiev: Kytys, 1999. Pp. 262 – 281, 286-287. 6. Histology: Textbook / YI Afanasyev, NA Yuryna, E. F. et al Kotovsky; Ed. YI Afanasyeva, NA Yurynoy. - 5th ed.,M.: Medicine, 1999. - Pp. 410-422. 7. Pathological physiology: (textbook-by for medical students. ) / NN Zaikov, Y. Beac, AV Ataman et al. - 3 rd ed.,-K.: "Logos", 1996. - Pp. 394 – 423, 196-223. 8. Lectures on the faculty of Pediatrics. 9. VD Chebotarev, VG Maidannyk Propaedeutic Pediatrics. - K., 1999. - Pp. 358 - 386. 10. Pediatrics: Textbook for students of higher medical educational institutions III-IV levels of accreditation. 2 edition revised and enlarged. / VG Maidannik. - Kharkov: Folio, 2002. - pp. 162-261. Rheumatic Diseases (guide for physicians), ed. VA Nasonova, NV Bunchuk. -M. , Medicine, 1997.- pp.257-304. 11. Selected issues of child cardiorheumatology. Manual for students of higher educational institutions of IV level of accreditation. / Ed. prof. AP Volosovec, VM Sava, SP Nick. - Kyiv, Kharkiv. - 2006. - pp. 7-35, 108-121, 157-175. 12. Children's Diseases (VM Sidelnykov, V. Berezhnaya, B. J. Resnik, etc.). - K.: "Health", 1999 - pp. 472 - 478. 13. Rheumatology childhood / under general ed. prof. EV Prokhorov, AP Volosovec. - Donetsk, 2006. - pp. 7-24. Additional 1. Derek S. Wheeler, Hector R. Wong and Thomas P. Shanley Cardiovascular Pediatric Critical Illness and Injury, 2nd ed. 2009. 2. Illustrated Textbook of Paediatrics/ Ed.: T. Lissauer, G. Clayden.- Edinburg: Mosby, 2006.- 410 pp. 3. Forfar & Arneil’s Textbook of Pediatrics/ Ed.: N.Mc Intosh, P.J. Helms, R.L. Smyth, S. Logan.- Edinburg: Churchill Livingstone, 2008. - 1687 pp.

The methodical instructions are compiled by Bubyr L.M.

Methodical instructions were discussed and approved at the meeting of department of Pediatrics №2 protocol № ___ of ______20____.

Additions and changes were made “____” ______20____ Additions and changes were made “____” ______20____ Additions and changes were made “____” ______20____ Additions and changes were made “____” ______20____ Additions and changes were made “____” ______20____ MINISTRY OF HEALTH OF UKRAINE UKRAINIAN MEDICAL STOMATOLOGICAL ACADEMY DEPARTMENT OF PEDIATRICS №2

Approved at the meeting of department of Pediatrics №2 Minutes № 21 " 05 " 06 2020 Head of the department MD, Prof. Kryuchko T.O. ______

METHODICAL INSTRUCTIONS FOR INDEPENDENT WORK OF STUDENTS DURING THE TRAINING FOR THE PRACTICAL (SEMINARS) CLASS

Academic discipline Pediatrics Моdule № 1 Content mоdule № 2 Non-rheumatic carditis and cardiomyopathy in children. The topic of the studies Infectious endocarditis. Course (year) 4 Faculty Medical

Poltava 2020 1. Actuality of the topic. The concept of the “carditis” was introduced in the clinical pediatric practice relatively recently. It means the simultaneous inflammation of heart membranes of myocardium, endocardium and pericardium. Nonrheumatic carditis (NC) during infancy occurs fairly often. According to data of autopsy prevalence of NC among children population is higher, than among adult one, and the frequency of cardities makes 2.3 - 8% of fatal outcomes of children. 0.5% of children who were hospitalized in permanent establishments due to different reasons have NC. Among persons who have a viral infection, the frequency of cardities is 10-15%. Thus frequency of cardities increases during the epidemics of viral infections. The annual incidence is just over 1 per 100,000 children, being greater in males than in females and in infants younger than 1 yr. The prevalence of cardiomyopathy in the newborn period is 10/100,000 live births, whereas for all children the prevalence is 36/100,000 for dilated cardiomyopathy and 2/100,000 for hypertrophic and restrictive cardiomyopathy. A concept “cardiomyopathy” was firstly used in 1957 for denotation of group of myocardium diseases of a not clear etiology. Heart failure is one of the most frequent complications of diseases of myocardium, which influence on quality of life and prognosis of disease.

2. The specific aims: - To define the etiologic and nosotropic factors of carditis in children. - To classify and to analyze the typical clinical manifestation of carditis in children. - To learn how make up the plan of examination and to analyze information of laboratory and instrumental investigation by typical course of carditis in children. - To demonstrate the knowledge of principles of treatment, rehabilitation and prophylaxis of carditis in children. - To make provisional diagnosis in the presence of carditis in children. - To carry out the prognosis of life at carditis in children. - To classify and analyze the typical clinical picture of cardiomyopathies. - To make the plan of laboratory and instrumental inspections at the typical flow of cardiomyopathies and heart failure in children. - To make a preliminary diagnosis at cardiomyopathies and estimate the degree of heart failure in children. - To demonstrate a domain principles of treatment, rehabilitation and prophylaxis of cardiomyopathies and heart failure and the first aid at the heart failure. - To carry out the prognosis of life at cardiomyopathies in children. - To demonstrate a domain moral and deontological principles of medical worker and principles of professional deference to the rank in cardiology of child's age.

3. Basic knowledge, skills, habits necessary for studing of the topic (inter-disciplinary integration) The names of the The acquired skills preceding disciplines General anatomy To know the anatomic features of the cardiovascular system of children General physiology To have the notions of physiological processes which take place in the cardiovascular system of children. Physiopathology To identify pathological processes which arise up at cardiovascular system on the basis of detected symptoms Microbiology with Knowledge of current classification of microorganisms and virology and immunology microbiological diagnostics. Nursing care and nurse To demonstrate the working knowledge of care for children with practice pathology of the cardiovascular system and rendering of first medical aid. Propedeutics of pediatrics Working knowledge of obtaining of anamnesis, clinical examination of a child, knowledge about basic clinical symptoms of children with pathology of the cardiovascular system, knowledge about the laboratory and instrumental methods of examination. Social medicine and Knowledge about the structure of rendering of medical and sanitary organization of health help to children population for the proper use of resources of the protection system of health protection on target of treatment and prophylaxis of origin of cardiovascular system diseases. Pharmacology knowledge about the main groups of medicinal preparations which are used in the treatment of cardiovascular system diseases of children.

4. The tasks for independent work during the training for the studies. 4.1. The list of basic terms, parameters, characteristics, which the student should master while training for the studies: Term Definition Nonrheumatic Is infectiously-inflammatory disease of heart during which myocardium, carditis endocardium and pericardium are, as a rule, simultaneously involved into pathological process. Myocarditis Affection of the cardiac muscle of an inflammatory character, which is conditioned by the action of infection, of parasitogenic or protozoa invasion, of physical or chemical factors, and which also arises up along with allergic, autoimmune diseases and transplantation of the heart. Idiopathic The heaviest and the most acute form which attends the deep diffuse myocarditis affection of a cardiac muscle (Abramov Fidler). Cardiomyopathies Diseases of myocardium which are related to cardiac dysfunction

Hypertrophic is characterized by abnormal ventricular hypertrophy of varying degrees cardiomyopathy (most commonly the ventricular septum is disproportionately involved compared to the left ventricular free wall) Dilated is characterized by varying degrees of dilatation of the ventricles, most cardiomyopathy prominently the left Restrictive is characterized by violation of tensility and relaxation of heart, that results cardiomyopathy in diastole dysfunction and by absence of ventricle hypertrophies Heart failure Diminishing of the cardiac troop landing in investigation of decline of pumping function of myocardium or worsening of his diastole weakening.

4.2. The theoretical questions to the studies: 1. Determination of the following notions: myocarditis, endocarditis, pericarditis, nonrheumatic carditis and cardiomyopathies. 2. Etiology of nonrheumatic carditis and cardiomyopathies in children. 3. Classification of nonrheumatic carditis and cardiomyopathies in children. 4. Pathogenesis of nonrheumatic carditis and cardiomyopathies in children. 5. Clinical picture of nonrheumatic carditis and cardiomyopathies in children. 6. Criteria of diagnostics nonrheumatic carditis and cardiomyopathies in children. 7. Treatment, prophylaxis and prognosis nonrheumatic carditis and cardiomyopathies in children.

4.3. The practical works (tasks) which are done at the studies: 1. Test paper. 2. Work of students with inflammatory heart diseases children: - collection of complaints, anamnesis of disease; - objective examination of children; - formulation of diagnosis in accordance with current classification; - to work out a plan of examination of a sick child; - to work out a plan of treatment of a sick child. 3. Clinical analysis of a typical case. 4. Solving of situational tasks.

5. The contents of the topic:

Contents of topic is exhaustively expounded in the following sources: lectures, educational and scientific literature, information in the Internet network.

Сarditis is a group of the inflammatory diseases of the heart predominantly affecting myocardium. Carditis is the disease of infectious and allergic origin. These lesions are caused by various infectious agents, predominantly by viruses.Сardites are difficult for diagnosing. Their prevalence in the children exceeds that in the adults. In the children with the syndrome of the sudden death within the first year of the life, the prevalence of the cardites amounts to 17%. In the children of the school age, the prevalence of the cardites is about 21%. No more than 55-60% of the children recover after carditis. In 30% of the patients, cardiodilatation remains, while in 15-20% of the patients myocardiofibrosis persists. The mortality rate in carditis assessed in different studies varies from 2.2% to 11.4%. The course of the disease is polymorphous. The latent and low symptomatic as well as severe and recurrent forms are recorded. The tendency for the transition to the chronic form of the disease is evident. Since carditis is resistance to the treatment, this pathology is the topical problem in the pediatric practice. ETIOLOGY The causes of myocarditis are diverse and include infectious, toxic, and autoimmune etiologies. Infectious etiologies, particularly viral, are most common in children. The most common causes of viral myocarditis are enterovirus (coxsackie group B), adenovirus, parvovirus B19, Epstein-Barr virus, cytomegalovirus, and human herpes 6 (HHV-6). Cases may be sporadic or epidemic, and have seasonal and geographical variation. Rarely, pediatric myocarditis may be associated with autoimmune disorders and drug hypersensitivity. The etiology and pathogenesis of myocarditis are presented in greater detail separately. Classification of cardites According to the time of Congenital (antenatal): early, late origin Acquired Etiology Viral, viral-bacterial, bacterial, parasitogenic, mycotic, caused by Yersinia sp., allergic (iatrogenic, postvaccinal, serum- mediated), idiopathic Predominant location of the Carditis (myo-, endo- or pericarditis or their combination). lesion Lesions affecting the conducting system of heart Course of the disease Acute (up to 3 months), subacute (up to 18 months), chronic (more than 18 months): recurrent, primary chronic (congestive, hypertrophic, restrictive variants) Severity Mild, moderately severe, severe Form and grade of the heart Left-ventricular, grade I, IIA, IIB, III insufficiency Right-ventricular, grade I, IIA, IIB, III Total Outcome and complications Cardiosclerosis, hypertrophy of myocardium, disorders of rhythm and conduction, pulmonary hypertension, lesions of the heart valves, constrictive myopericarditis, thromboembolic syndrome

The grades of the heart insufficiency in cardites in the children Grade Right-ventricular insufficiency Left-ventricular insufficiency I The signs of the insufficient circulation at The same rest are not evident and appear only after the physical activity manifesting as tachycardia or dyspnea, the liver size is within the normal values IIA Heart rate increases by 15-30% per minute, The liver extends from the edge respiration rate increases by 30-50% per of the costal arch by 2-3 cm minute as compared to the physiological values IIB Heart rate increases by 30-50% per minute, The liver extends from the edge respiration rate increases by 50-70% per of the costal arch by 3-5 cm. The minute as compared to the physiological pastosity is observed. The values. The obtrusive cough, the bubbling jugular venous distention may be rales, and acrocyanosis are possible recorded. III Heart rate increases by 50-60% per minute, Hepatosplenomegalia, , respiration rate increases by 70-100% per hydrothorax minute as compared to the physiological values. The characteristic clinical conditions of pre-edema and edema of lungs are evident

The criteria of diagnosing carditis in the children The signs of high The signs of medium significance The signs of low significance significance (scored (scored as 2 points) (scored as 1 point) as 4 points) Increased size of the The absence of the signs indicating Heart pain. heart or its cavities. the control of heart functions by the Diminution of the intensity of Decreased autonomic nervous system. the heart sound I contractility of The detection of the cardiac antigen Tachycardia or bradycardia. myocardium. and anticardial antibody in the The gallop rhythm. blood. The apical systolic murmur. The increased blood level of Sinoauricular blockade. cardiospecific fraction of isozymes: The impairment of the lactate dehydrogenase, malate atrioventricular conduction. dehydrogenase, creatine The ectopic rhythm. phosphokinase, etc. Extrasystoles. ECG signs of heart hypertrophy. The displacement of S-T ECG signs of heart ischemia. interval The alteration of T wave.

PATHOLOGY At gross examination, the myocardium appears pale and flabby. The ventricular cavities are dilated, with wall thinning and an increase in myocardial mass. A mural thrombus is occasionally present. Microscopic inspection demonstrates a predominantly mononuclear inflammatory cell infiltrate. Interstitial edema and, in more severe cases, myocyte necrosis are present. PATHOPHYSIOLOGY The primary pathophysiological processes are the development of myocardial necrosis and interstitial edema, with consequent impairment in the contractile performance of the heart muscle. Chamber dilation and thinning of the ventricular wall increases wall tension, augments myocardial oxygen demand and stimulates hypertrophy. Preload reserve is minimal in the immature myocardium, and the maintenance of an adequate cardiac output in the face of failing contractility is dependent on chronotropic upregulation. Comorbid fever and anemia can place further stress on the heart. When compensatory mechanisms are depleted, the heart fails, resulting in a low output state. A decrease in cardiac compliance, along with incomplete emptying of the left ventricle, raises left ventricular diastolic and pulmonary venous pressures, and predisposes patients to pulmonary edema. The diagnosis of the carditis is regarded as the reliable one when the sum of the scores is 5 points and more (with at least one sign of high or medium significance being present). The diagnosis is regarded as the probable one when the sum of the scores is 3 points. The peculiar features of the various variants of carditis in the children Acute carditis develops directly at the time of viral infection or the infection of other etiology or soon after such infection, often in the children with the non-favorable premorbid state (allergic or lymphatic diathesis; in the children with recurrent diseases). The severe forms are observed predominantly at the age below 3 years. The forms of the moderate severity may be observed both in the children of early age and in preschool and school age children. The mild forms are recorded predominantly in preschool and school age children. Subacute carditis is characteristic of the preschool and school age children. The signs of the heart insufficiency become evident in 4-6 months after the acute respiratory viral infection. At the first stage, the clinical manifestation may be insignificant. Then the following signs become evident: the cardiac hump, the systolic murmur of mitral valve insufficiency, the persistent accent of the second sound over the pulmonary artery. The treatment-resistant cardiac insufficiency develops. Chronic carditis occurs in the children of the school age. The primarily chronic carditis with the clinically asymptomatic onset is possible. Otherwise, the chronic carditis may be a consequence of the acute or subacute process. The congestive or dilatation carditis, the hypertrophic carditis and the restrictive carditis with the drastically decreased volume of the left ventricle may be distinguished as the clinical variants. Since the heart insufficiency develops later in the course of the disease, such condition is often missed for being diagnosed early. Clinical manifestations of non-rheumatic myocarditis. The extracardial signs of the heart lesion become evident: the decreased appetite, the flabbiness, the anxiety with groaning in the night, the irritability, the nausea, and the vomiting. The signs of the congestive cardiac insufficiency are also apparent: the cough, which increases upon the change of the body position, the cyanosis attacks, the dyspnea; the rales in the lungs (the asthmatic components in the left-ventricular insufficiency), the increased liver size (in the right-ventricular insufficiency), the pastosity of the tissues, the decreased diuresis. The apical beat is weakened, the borders of the heart are moderately broadened, the tachycardia appears, the first sound over the apex is muted, the gallop rhythm is possible as well as tachycardia, bradycardia, tachyarrhythmia or bradyarrhythmia. The following variants of the course may be distinguished: low-symptomatic, pseudocoronary (painful), decompensated (with impaired blood circulation), arrhythmical, pseudovalvular (with the signs of the dysfunction of the heart valves, often mitral valve), thromboembolic, and mixed. The peculiar features of endocarditis in the children. Frequently, the onset of the disease is without peculiar symptoms except for the rapid fatigability. The low febricity, the general weakness is possible. The classic signs of the endocarditis may be absent. Among the characteristic features are cardio- and splenomegaly, petechias, the loss of body mass, the finger deformities in the shape of the drumstick fingers. If the adequate treatment has not been provided, the retinal hemorrhages (Roth spots), the erythematous painful spots in the palms and the soles, and the painful nodules in the skin in the finger cushions (Osler's nodules) develop. The peculiar features of pericarditis in the children. For the pericarditis the following signs are typical: the pain in the left shoulder and the back, which decrease in sitting position, the frequent fevers, tachypnea, tachycardia, the cough, the muted heart sounds due to the pericardial effusion, the pericardial friction rub. The extensive effusion may be a cause of the heart tamponade. The jugular venous distention in the course of breath-in may be recorded with the paradoxic pulse (the decrement in the arterial blood pressure in the breath-in exceeds the physiological values, while the decrement in the venous blood pressure in the breath-in is lower than the corresponding physiological values). The peculiar features of congenital carditis in the children. The intrauterine carditis may be detected antenatally. Nevertheless, more frequently this condition is diagnosed within the first postnatal weeks or months. According to the term of the origin, early and late carditis may be delineated. The early carditis originates on the months 4th-7th antenatally and is manifested by the growth of the fibrous tissue in the subendocardial layers of myocardium resulting in fibroelastosis of endocardium or the predominant growth of the elastic tissue resulting in elastofibrosis of endo- and myocardium without the overt features of the inflammation. The first symptoms of the disease that become evident in the first postnatal months comprise the decrease appetite, the delay in the physical development, the flabbiness, the paleness of the skin and the mucosa, the fatigability in the course of the sucking, the extension of the heart borders, the early development of the cardiac hump, the muted heart sounds. The coarse murmurs are not detectable. Nevertheless, sometimes the systolic murmur of the relative mitral insufficiency may be heard. The insufficiency of the left ventricle, which is resistant to the treatment, prevails. The ECG findings are such as follows: the rapid rhythm, the high voltage of QRG complex, the hypertrophy of the left ventricle. On X-ray pictures, the shape of the heart in spherical in the case of the fibroelastosis and trapezoid in the case of the elastofibrosis. The late carditis originates after the 7th month of the antenatal development. This form is characterized by the substantial inflammatory alterations in myocardium involving two or three heart envelopes, the conducting system, sometimes the coronary vessels with the resulting sclerosis of the blood vessels and the hypertrophy of myocardium without the growth of the elastic and fibrous tissue. When the cords and the valves of the heart are involved into the process, the congenital heart disease develops. The clinical and instrumental symptoms of the disease are similar top that in the severe form of the chronic non-rheumatic carditis with the delay in the gain of body mass after 3-5 months of the postnatal life, the fatigability during the feeding, the sweating. The apical beat is slightly enhanced, the borders of the heart are extended moderately, the heart sounds are clear, the murmurs are not heard, the cardiac insufficiency is less pronounced as compared to the early carditis. Tachy- and bradycardia, bradyarrhythmia are detectable. In ECG: arrhythmia, atrioventricular blockade, the overloading of the left ventricle and the atria. X-ray findings: the shadow of the heart increased less than in the variant described above. Idiopathic myocarditis (Abramov-Fidler) represents the most severe acute form of the disease accompanied by the deep diffuse lesion of the myocardium. The following features are typical for this condition: the pronounced intoxication syndrome, cardialgia, dyspnea, cardiomegalia with the relative insufficiency of the mitral valve (less frequently other valves are also involved), the sounds are muted significantly, and ECG pathology varies. Sometimes the cardiac fibrillation is evident. The circulatory insufficiency may progress and the thromboembolic syndrome may develop. According to the predominant symptoms, the following variants may be distinguished: stenocarditic, arrhythmic, asystolic, and thromboembolic. The laboratory findings usually are not changed. The prognosis is unfavorable and most patients die. In many cases, the use of prednisolone is advantageous for attaining the significant improvement (starting from 30-50 mg daily followed by the decrease in the daily dose). The pathogenetic therapy is performed taking into account the clinical variant of the disease. DIFFERENTIAL DIAGNOSIS Sepsis, severe dehydration or anemia should be considered as diagnostic possibilities in the ill neonate or infant. Primary endocardial fibroelastosis and metabolic dilated cardiomyopathy are myocardial disorders that can present in a similar fashion. Congenital structural lesions, such as critical coarctation of the aorta or anomalous origin of the left coronary artery from the pulmonary artery, must be ruled out. Supplementary techniques for diagnosing carditis in the children Laboratory examinations The most reliable technique for confirming the diagnosis of the acute non-rheumatic carditis in the children consists in detecting the infectious agent in the blood, the nasopharyngeal mucus, and the feces as well as identifying the high titers of the corresponding antibodies in the paired sera of the patients (four-fold increment in 2-4 weeks) followed by the decrease in the titer. The changes of the parameters of the general blood test and the biochemical parameters of the blood such as the increased ESR, leukocytosis or leucopenia, the increased content of alpha-2 and gamma-globulins, C-reactive protein, DPA assay are associated with the viral or bacterial infection. In most cases including those with the severe course of the disease with cardiomegaly, the activities of the organ-specific enzymes (creatine phosphatase, lactate dehydrogenase) remain unchanged. Electrocardiography. The following ECG signs may be detectable for diagnosing non- rheumatic carditis in the children: the impaired conduction (the atrioventricular blockade grade II-III, the blockade of Gis bundle), extrasystoles, the paroxysmal tachycardia with the extended and deformed QRS complex, the alterations in ST segment, the inversion of T wave, the hypertrophy of the chambers of the heart. X-ray examination. The following signs are detectable: the extension of the heart borders with the cardiac index exceeding 50, the alteration of the contours of the heart and the large vessels, the increased pulmonary X-ray pattern. Echography of the heart This technique allows one for detecting the hypertrophy of the heart chambers, the dilatation of the heart cavities. Doppler echography is the useful technique for assessing the state of the valves of the heart. Treatment of carditis in the children The treatment depends on the etiology of the disease, the peculiar features of the immune reactivity of the child, the course of the disease, and the grade of the cardiovascular insufficiency. The treatment comprises the hospital period (acute disease or the acute condition of the disease), the outpatient period, and the sanatorium treatment period (the maintenance treatment). Principles of the hospital treatment of carditis in the children 1. The limitation of the movements for 2-4 weeks. The treatment exercises are performed after the decrease in body temperature with the exercise load selected according to the results of the functional tests (after Shalkov). 2. The diet demands the limitation of the volume of liquid (the daily liquid consumption should be inferior to the diuresis volume by 200-300 mL). The ration should comprise the food rich in potassium such as the raisins, the dried apricots, and the baked . 3. Antiviral and antibacterial therapies are provided. The duration of the antibacterial therapy should be not less than 3-4 weeks. 4. The antibacterial therapy with Penicillins is performed on the Weeks 2-3 of the hospital treatment as to eliminate the chronic infectious foci present in most patients with carditis. 5. The non-steroid anti-inflammatory drugs are prescribed for 4-6 weeks in the acute carditis (Indometacin at a dose 1-2 mg per 1 kg of body mass; Voltaren at a dose of 0.5 mg per 1 kg of body mass daily). Then the drugs are used for further 2-3 months at a half-dose. In the cases of the subacute course or the recurrence of the chronic carditis, the full doses of the drugs stated above are prescribed for 6-8 weeks. 6. Glucocorticoids are indicated in the severe course of the disease with the signs of the cardiac insufficiency (often in the young children and in the children of the school age with Abramov-Fidler myocarditis), in the cases involving the conduction system, and when the danger of the transition to the chronic course of the disease is significant. Prednisolon is prescribed at a dose of 1-1.5 mg per 1 kg of body mass daily for one month followed by the gradual dose reduction by 2.5 mg for 3-4 days in the children of the school age and 1.2-1.5 mg for 3-4 days in the young children. When the effect is insufficient, the maintenance dose of 0.5 mg per 1 kg of body mass daily is provided. 7. In the case of the cardiac insufficiency, the diuretics are used daily for 1-1.5 months. In the case of the left-ventricular insufficiency grade I-IIA, Veroshpiron is used at a dose of 1-4 mg per 1 kg of body mass daily; in the case of the left-ventricular insufficiency grade IIA with the concomitant right-ventricular insufficiency grade Iia-IIB, Verospiron is used in the combination with Furosemid (2-4 mg per 1 kg of body mass daily); in the case of the total insufficiency grade IIB-III, Lasix or Furosemid are administered parenterally in combination with Verospiron; when necessary Brinaldix or Uregit (1-2 mg per 1 kg of body mass daily) are used. Upon the discharge from the hospital, the drugs are taken 2-3 times a week. 8. The desensitizing drugs are indicated only in the acute or subacute course of the carditis. 9. The therapy is administered in the cases of the thromboembolic variant of the disease: Heparin is used at a dise of 120-150 U per 1 kg of body mass; Curantil or Dipiridamol is used as a dose of 5 mg per 1 kg of body mass daily. 10. Antikinin therapy is provided in the cases of the utmost acute course of the disease (Parmidine, Arginine, Predectin, Contrical). 11. In the cases of cardiac insufficiency, the cardiac glycosides are provided. 12. Cardiometabolic drugs are used intravenously as the polarizing mixture (10% glucose solution –10-15 mL per 1 kg of body mass, 1 U of insulin per 5 g of glucose, Panangin – 1 mL per each year of the life, 2-5 mL of 0.25% solution of Novocain). Riboxin is used per os for one month. In addition, the following drugs are used: potassium orotate, Panangin, vitamin B12 with folic acid, calcium pangamate (vitamin B15), calcium pantotenate (vitamin B5), L-carnitine or Mildronate, non-steroid anabolic hormones (Retabolyl, Nerobol, Femobolyn, Metandrostendiol). 13. Antiarrhythmic drugs are used depending on the form of arrhythmia. 14. In the subacute or chronic course of the disease, the drugs belonging to Aminoquinolines are indicated: Delalgil, Plaquenil (3 mg per 1 kg of body mass daily) for a long period, 3-6 months and more. Principles of the treatment of cardiac insufficiency. The acute cardiac insufficiency is the insufficiency of blood circulation due to non-efficient pumping ability of the heart resulting in decreasing cardiac output or inability to pump the whole venous inflow per unit of time. Clinically, the acute cardiac insufficiency is manifested as the syndrome of low cardiac discharge. The following elements of the therapy of cardiac insufficiency are to be followed: The adequacy of the venous inflow to the heart should be provided. This aim is attained by the use of diuretics (Lasix intravenously at a dose of 1-2 mg per 1 kg of body mass for one injection, 2-3 injections a day) and the respiratory therapy with breathing in the oxygen with the positive pressure at the end of expiration. The improvement of the inotropic activity of myocardium, in other words – the increase in the strength of heart contractions. This aim is attained by the use of adrenomimetics of fast action (Dopamine, Dobutamine, Adrenalin hydrochloride). In the case of the supraventricular tachycardia in the congestive cardiac insufficiency, Digoxin is used intravenously at a dose of 0.03-0.05 mg per 1 kg of body mass. In tachyarrhythmia, the drugs for the normalization of heart rhythm are provided (Quinidine, Novocainamde, Isoptin, Anaprilin, Cordaron, Ornid, Verapamil, etc.). In bradyarrhythmia, the drugs, which do not affect the heart rhythm, are prescribed (Adonis, Taluzin). The cardiotrophic drugs are used (potassium and magnesium-containing drugs, pyridoxal phosphate, Phosphaden, ATP-long, Riboxin, Inosin-F). Vasodilating drugs are also advantageous for decreasing the venous inflow to the heart due to the decrease in the peripheral resistance of blood vessels. With this aim in view, Nanipruss (Nitroglycerin) is administered in microstream mode or ACE inhibitors such as Captopril or Enalapril are used (1-2 times a day at a dose 0.5-0.6 mg per 1 kg of body mass for the patients aged below 3 years and 12.5 mg for the patients of the preschool age). The regular medical check-up of the children with carditis (outpatient and sanatorium phases) Following the acute carditis, the child should be observed on the outpatient basis by cardiorheumatologist for 5 years. Upon the discharge from the hospital, the child should be examined monthly for three months, then one time in three months for further one year, and then twice a year with the obligatory record of ECG. Once or twice a year, X-ray examination of the chest is performed. At this phase of the treatment, the drugs improving the myocardial metabolism are used 2-4 courses every year (potassium orotate, Panangin, vitamin B12 with folic acid, calcium pangamate, calcium pantotenate, L-carnitine or Mildronate), non-steroid anabolic hormones (Retabolyl, Nerobol, Femobolyn, Metandrostendiol). When indicated, the cardiac glycosides are prescribed at the maintenance doses. In addition, antiarrhythmic drugs, diuretics are used 1-3 times a week. Aminoquinolines are used for 3-6 months. The are administered for preventing the stress, which may result in cardiogenic shock. In the case of the acute intercurrent diseases, the non-steroid anti-inflammatory drugs for 2-3 weeks are useful. Bicillin is used in the cases of the chronic tonsillitis for one year. The question on whether tonsillectomy should be performed in resolved on an individual basis. The child should be exempted from the physical exercises at school except for graduated walking. The vaccination is performed one year after the acute process has calmed down. At the same term, the sanatorium treatment is indicated. When the disease becomes chronic, the child should remain at the dispensary registry and the regular examinations are performed once a month. Once in three months, ECG should be recorded. Once or twice a year, X-ray examination of the heart is performed. When cardiosclerosis develops, the child also should remain at the dispensary registry and the regular check-ups are performed 3-4 times a year. For the patients with the cardiac insufficiency, the physical and psychic loads should be limited. The scope of the treatment depends on the individual course of the disease. The planned vaccination is not performed. Pediatric cardiomyopathies are disorders that affect the muscles of the heart. At any given time point, they affect at least 100,000 children worldwide. As a chronic disease, pediatric cardiomyopathy requires a comprehensive treatment approach and a multidisciplinary chronic disease program approach in order to improve health outcomes in a validated, cost-effective manner. The course of pediatric cardiomyopathy is often progressive. The identification of risk factors helps define high-risk populations and leads to early diagnosis and therapy, which may alter the disease course. It is more commonly diagnosed in younger children with the average age at diagnosis being 2 years. Dilated cardiomyopathy can be familial (genetic), and it is estimated that 20–30% of children with DCM have a relative with the disease, although they may not have been diagnosed or have symptoms. Cardiomyopathy may also be termed primary or secondary. Primary cardiomyopathy occurs by itself or for unknown reasons (idiopathic). Secondary cardiomyopathy occurs secondary to a known cause such as heart muscle inflammation (myocarditis) caused by viral or bacterial infections; exposure to certain toxins such as heavy metals or excessive alcohol use; or certain disorders that affect the heart in addition to additional organ systems. According to the Pediatric Cardiomyopathy Registry, approximately 79 percent of pediatric cardiomyopathy is idiopathic. Cardiomyopathy has traditionally been divided into three basic subtypes based upon the specific changes within the heart. These subtypes are: dilated, hypertrophic, and restrictive. There are several other less common forms such as arrhythmogenic right ventricular dysplasia, noncompaction, and others where it is controversial whether they are subtypes of the three principle forms or should be considered as completely different diseases. Signs and symptoms. Dilated Cardiomyopathy Dilated cardiomyopathy is characterized by abnormal enlargement (dilatation) of the left and/or right ventricle because of a weakening of the heart’s pumping action, causing a limited ability to circulate blood to the lungs and the rest of the body which may result in fluid buildup in the lungs and various body tissues, a condition known as congestive heart failure. In some individuals, all four chambers of the heart may be affected. Symptoms of congestive heart failure may depend upon an affected child’s age and other factors. In young children, for example, heart failure may be manifest as fatigue and shortness of breath (dyspnea) upon exertion. Additional symptoms may include swelling of the legs and feet and, in some people, chest pain. Initial symptoms of dilated cardiomyopathy in infants and children may include irritability, a persistent cough, shortness of breath, and poor feeding resulting in the failure to gain weight at the expected rate (failure to thrive). Affected individuals may also experience excessive sweating, fatigue, wheezing, and paleness of the skin (pallor). More serious complications may include fainting episodes (), abdominal pain, irregular heartbeats, and fluid accumulation within the lungs (pulmonary congestion) resulting in a persistent cough. Hypertrophic Cardiomyopathy Hypertrophic cardiomyopathy is characterized by abnormal thickening of the walls of the heart potentially resulting in obstruction of blood flow in and out of the heart. In most patients, the left ventricle is affected. The symptoms of hypertrophic cardiomyopathy vary widely among affected individuals. In many cases, affected individuals have no symptoms. Affected infants and children may experience shortness of breath upon exertion, fatigue, excessive sweating, and poor appetite and weight gain resulting in growth failure. As affected children age, they may experience chest pain or discomfort, irregular heartbeats, dizziness or fainting episodes (syncope) usually upon heavy exertion, and, eventually, congestive heart failure and fluid accumulation within the lungs. In some cases, affected individuals may experience sudden cardiac arrest and, potentially, sudden death. Restrictive Cardiomyopathy Restrictive cardiomyopathy is extremely rare in children. In this form of cardiomyopathy, the muscular walls of the heart become stiff, impeding blood flow into the heart. Symptoms associated with restrictive cardiomyopathy in infants and children include shortness of breath, fatigue, chest pain, and poor appetite and weight gain, resulting in growth failure. Additional symptoms may include fluid collection in the abdomen (ascites) and feet due to accumulation of fluid, congestion of the lungs, and an abnormally large liver (hepatomegaly). Irregular heartbeats, the formation of blood clots and heart block may also occur. Restrictive cardiomyopathy may progress to cause congestive heart failure and sudden death. Diagnosis Pediatric cardiomyopathy may be diagnosed based upon a thorough clinical evaluation, identification of characteristic physical findings, a complete patient and family history, and a variety of specialized tests. Such tests may include x-ray studies (e.g., computed tomography), electrocardiography (EKG), or echocardiography. An EKG, which records the electrical activities of heart muscle, may reveal abnormal electrical patterns (e.g., resulting in arrhythmias). During an echocardiogram, sound waves are bounced off the heart (echoes), enabling physicians to study cardiac function and motion. Three additional tests that may be performed for evaluation of heart disease are cardiac catheterization, cardiac magnetic resonance imaging (MRI) and radionuclide ventriculogram. During the cardiac catheterization, a small hollow tube (catheter) is inserted into a large vein and threaded through the blood vessels leading to the heart. Cardiac catheterization may enable physicians to withdraw blood to assess oxygen content, measure blood pressure in the heart, evaluate heart function, obtain small samples of myocardial tissue for microscopic evaluation, or thoroughly identify certain anatomical abnormalities. Cardiac MRI generates images of the heart similar to echocardiography but uses magnetic waves instead of sound waves. During radionuclide ventriculogram, tiny amounts of low-dose radioactive materials (tracers) are injected into to a vein and travel into the heart. Tracers release energy that is used by special cameras to produce pictures of the heart. Because certain forms of cardiomyopathy may occur as part of a larger genetic disorder, infants and young children with a diagnosis of cardiomyopathy should receive specific tests to rule out any potentially associated disorders such as metabolic disorders. Complications of Hypertrophic cardiomyopathy: violation of rhythm of heart, arrhythmias; syncopal states; infectious endocarditis, the most threatening complication is an sudden death. Treatment: – to limit the physical loadings; – β-blockers (Propranolol 0,5-2 mg/kg/24 hr); – if necessary Verapamil 4-10 mg/kg/24 hr; – prophylaxis of infectious endocarditis; – at violation of cardiac rhythm is conformable treatment; – treatment of heart failure – at an obstructive form - rotined cardiac surgery treatment. Complications of Dilated cardiomyopathy: heart failure, violations of rhythm of heart, arrhythmia, thromboembolia, infectious endocarditis. Treatment: – specific treatment is not present; – therapy of stagnant heart failure - therapy of dysrhythmia – a prophylaxis of thrombosis with direct and indirect ; – transplantation of heart. Standard Therapies The treatment of pediatric cardiomyopathy is directed toward the specific symptoms that are apparent in each individual. Such treatment may require the coordinated efforts of a team of medical professionals, such as pediatricians; physicians who specialize in childhood heart disease (pediatric cardiologists); specialists in the study of the blood and blood-forming tissues (hematologists); pediatric cardiothoracic surgeons; physical therapists; occupational therapists; and/or other health care professionals. Individuals with pediatric cardiomyopathy may be treated by lifestyle changes, dietary restrictions, various medications, and surgery. It is important to note that many drug therapies and surgical techniques used to treat cardiomyopathy have predominantly been used and tested in adults. Only limited information exists detailing the effectiveness of such therapies in children with cardiomyopathy. More research is necessary to determine the long-term safety and effectiveness of such therapies in the pediatric population. Specific therapeutic procedures and interventions may vary, depending upon numerous factors such the specific type of cardiomyopathy present; the progression of the disease upon diagnosis; an affected individual’s age; associated health conditions; an individual’s tolerance to certain medications; and additional factors. Individuals with dilated cardiomyopathy may be treated with a variety of medications including drugs that reduce abnormal fluid retention by promoting the production and excretion of urine (diuretics); drugs that reduce the workload of the heart by blocking certain substances from binding to structures within the heart (beta blockers); and digitalis medications such as digoxin, which increase the efficiency of heart muscle contractions and produce a more regular heartbeat. Another type of medication used to treat individuals with dilated cardiomyopathy are vasodilators, which relax blood vessels, thereby lowering the blood pressure and minimizing the effort needed by the heart to pump blood throughout the body. Angiotensin-converting enzyme (ACE) inhibitors are a type of vasodilator. Children with a more serious dilated cardiomyopathy may need surgery to implant a device that helps maintain normal heart rhythm through electrical stimulation (pacemakers or defibrillators. If drug therapy fails some individuals may require a heart transplant (see below). Individuals with hypertrophic cardiomyopathy may be treated with a variety of drugs including beta-blockers, calcium channel blockers, and drugs that regulate irregular heartbeats (anti-arrhythmics). If drug therapy does not work, a permanent pacemaker or defibrillator may be implanted to help control irregular heartbeats. In some patients where drug therapy does not work, the blockage that causes the enlargement of the heart and restricts blood flow that characterizes hypertrophic cardiomyopathy may be treated with surgery. Surgical techniques may include septal myectomy or alcohol ablation. Septal myectomy is a type of open-heart surgery, in which a portion of the abnormally thick and stiff ventricular septum (the partition that separates the left and right ventricles) is removed. This procedure allows for improved blood flow and reduces the symptoms associated with severe hypertrophic cardiomyopathy. Alcohol ablation is a new catheterization procedure in which alcohol is used to destroy certain heart cells, thereby shrinking the heart muscle and resulting in improved blood flow. In some cases of hypertrophic cardiomyopathy, a heart transplant may ultimately be necessary (see below). Restrictive cardiomyopathy may be treated with diuretics and drugs that prevent blood clotting (anticoagulants). A pacemaker or defibrillator may be implanted to help control irregular heartbeats. In most cases, heart transplantation will be necessary. Some physicians recommend that affected children be listed for transplant as soon as the diagnosis is made. In many children with pediatric cardiomyopathy, the disorder progresses to the point where medications and surgical treatment options are ineffective. In such cases, affected children may require a heart transplant, a form of open-heart surgery in which a severely diseased heart is replaced with a healthy donor heart. Pediatric cardiomyopathy is the leading cause of heart transplantation in children. A heart transplant is considered a last resort for individuals with end- stage heart failure. Drawbacks of heart transplantation include the potential for rejections and the limited availability of a suitable donor. Genetic counseling may be of benefit for affected individuals and their families. Other treatment is symptomatic and supportive. Treatment of heart failure Acute left-type heart failure: 1. Position of a child in a bed with heaved up a head on 30°. 2. Oxygen therapy by 30-40% oxygen (at possibility combination with ethyl spirit) through a mask or nasal catheter. 3. Furosemid (Lasix) IV: 1-2 mg/kg to 4 times per day. 4. IV 10 % glucose, 4% chloride of calcium or Panangin (volume 2/3 of the age- dependent necessity). 5. IV Prednisolon: 3-5 mg/kg. 6. 2% Promedol IV: 0,1-0,2 mg/kg (0,05-0,1 ml/year). 7. Dobutamin IV in a dose 2-15 μg/kg/min. Side-effects: tachycardia, hypertension, violation of rhythm. 8. Correcting the fluid, acid-base and electrolyte balance. 9. Antibiotic for the prophylaxis of pulmonary complications and endocarditis. 10. Intubation of trachea at the threat of heart arrest and breathing. Acute right-type heart failure: 1. Oxygen therapy by 40-50% oxygen. 2. Furosemid (Lasix) IV slowly: 1-2 mg/kg to 4 times per day. 3. Correcting the fluid, acid-base and electrolyte balance. 4. Intubation of trachea and artificial ventilation at the cardiac arrest and breathing.

6. The materials for self-control A. Sample case report 1. A girl of 10 months was directed into hospital with complaints about flabbiness, excessive sweating, thirst, repeated vomits. The child is of IV pregnancy which progressed without features, II childbirths which were in time. The body weight at birth was 3900 g, body length - 53 cm. The child developed according to its age. At 8 months she was treated in the infectious department with the diagnosis of intestinal infection of agnogenic etiology. After discharge from the department a girl had weakness, bad appetite. The symptoms of intoxication increased. Repeated examination detected the extrasystole. The condition of the child is severe at the examination. The skin is pale with short-term hyperemia. Breathing rate is 40 per minute. Auscultatory above lungs the breathing is vesicular; in the lower parts there are fine moist rales. Heart sounds are muffled, irregular extrasystoles are frequent. The liver + 4 cm; slightly swollen. On the electrocardiogram – there is supraventricular extrasystole on the background of sinus rhythm frequent, up to 20 extrasystoles per minute. Signs of overload of both atrium and ventricles, insignificant decrease of exchange processes in myocardium. On the X-ray signs of cardiomegaly are absent. Task: 1. Diagnose and substantiate your diagnosis. 2. Make up the investigation plan. 3. Make up a plan of treatment. 4. Prescribe to this child digoxin intravenously.

2. A girl of 1.5 year, of II full-term pregnancy; on 25-th week mother had a viral infection. She cannot walk without help, sits with the support; deficit of the body weight makes 24%. The skin is pale, persistent acrocyanosis, which increases with the easy physical loading. Breathing rate is 50 per minute. There is muscular hypotonia. The thorax is deformed, a cardiac hump is forming. The heart borders are extended more to the left. Heart rhythm is unrhythmical, heart sounds are weakened, heart rate is 152 per minute. The liver +4 cm. Swollen legs. Electrocardiogram: rhythm is sinus, irregular, group ventricular extrasystoles are frequent, right atrium is overloaded, left ventricle is hypertrophied, myocardial hypoxia. 1. Diagnose and substantiate your diagnosis. 2. Make up the investigation plan. 3. Make up the treatment plan. 4. Prescribe to this child amiodaron.

3. A girl of 6 months is ill during 10 days. The disease began acutely with a high temperature and massive catarrhal manifestations. She received antipyretics. At the 4th day child felt herself better, a temperature fell down to that of low grade fever, the catarrhal manifestations were diminished. On the 8th day from the beginning of disease a child became week, sleepy, an appetite became worse. At the examination the child is pale, there is a shortness of breath and acrocyanosis. The heart borders: right one is on a right parasternal line, top one is on IInd rib, left one is on the front axillary line. Heart sounds are hyposthenic, inferior systolic murmur is above an apex. Heart rate is 180 per minute, is rhythmical, breathing rate is 55/minute. The liver + 3 cm and is elastic. 1. Diagnose and substantiate your diagnosis. 2. Make up the investigation plan. 3. Make up the treatment plan. 4. Prescribe to this child riboxin intravenously.

4. A boy of 11 year complains of frequent piercing pains in the region of the heart, palpitation, shortness of breath at the moderate physical loading, periodic increase of temperature up to that of low grade fever. In anamnesis there are frequent anginas respirators episodes, last time was ill with the viral disease 3 weeks ago. At the examination the general condition of a child is relatively satisfactory. The skin is pale-pink. Tonsils are loose, hypertrophic, there are carious teeth. The heart borders are within the limits of age-old norm, cardiac performance is rhythmical, heart sounds are weakened, inferior systolic murmur is above an apex. Arterial blood pressure is 100/50 mm Hg. Pulse is 92 per minute. The liver is not enlarged. There are no aberrations in the general analysis of blood. Electrocardiogram: rhythm is sinus, regular, moderate myocardial hypoxia. Phonocardiogram: systolic murmur of middle amplitude and middle frequency above an apex, unconnected with a 1st sound, on 1/3 of a systole. Consultation of ENT-doctor is chronic decompensated tonsillitis. 1. Diagnose and substantiate your diagnosis. 2. Make up the plan of examination. 3. Make up the treatment plan. 4. Prescribe to this child diclofenac.

5. A girl of 10 year was directed into hospital in the severe condition with complaints about fatigability, shortness of breath, heart pains, edema on legs, sensation of load in the right hypochondriac area. She is ill during 3 years, was repeatedly treated in permanent establishment, improvements are short-term. At the examination a child has semisitting position. Breathing is hypopnoe, noisy, up to 40 per minute. The skin is cyanotic and grey, cheeks are crimson, edema of feet and shins, cardiac hump. In the lungs there are multiple moist rales, mainly in inferior parts. The heart borders: right one is on a right midioclavicular line, top one is on II rib, left one is on the middle axillary line. An apex beat is increased, diffuse. Cardiac performance is rhythmical, cardiac sounds are weakened; slight systolic murmur is on the apex. The pulse is 120 per minute. A liver comes forward on 3 cm from under the edge of costal arc. Electrocardiogram: the rhythm is sinus, regular, tachycardia, disturbance of ventricular conduction; block of the right crus of His' bundle. 1. Diagnose and substantiate your diagnosis. 2. Make up the plan of examination. 3. Make up the treatment plan. 4. Prescribe to this child furosemid intravenously.

6. A mother with a girl of 5 years complaints of that the child has become irritable, the appetite has decreased; cough, dyspnea, cyanosis after walking and running concern. OBJECTIVELY: the pale skin, the enlarged borders of the heart, the pulse is 120 beats/min, weak; weakened heart sounds, gallop rhythm, over the lungs there are moist rales in the lower parts. General blood test is in norm. X-ray: the heart is enlarged. 1. Put a preliminary diagnosis. 2. What diseases should be differentiated by the diagnosis? 3. Make up the investigation plan. 4. Write a prescription for furosemid.

7. A boy of 10 complains of periodic squeezing pain in the heart, sudden attacks of weakness. On the electrocardiogram there were revealed signs of left ventricular hypertrophy. On the echocardiography - asymmetric hypertrophy of the ventricular septum. 1. Put a diagnosis. 2. What diseases should be differentiated by the diagnosis? 3. What treatment tactics should be in this case? 4. Write a prescription for verapamil.

8. A girl of 13 lost consciousness during a lesson. The school doctor found that similar conditions have been repeating since recently, the child feels sharp weakness, dizziness. No Rheumatism in the anamnesis. According to the girl, in the family there have been cases of death from heart disease. Objectively: extended left and lower heart borders, on the apex of the heart there heard systolic murmur, which is not conducted and increases the during Valsalva test. 1. Put a preliminary diagnosis. 2. Make up the investigation plan. 3. What treatment tactics should be in this case? 4. Write a prescription for propranolol.

9. A boy of 11 complains of shortness of breath, coughing, edema of shins when walking, and shortness of breath after a slight physical exertion. Previously has not been ill. The disease has developed gradually. Objectively: acrocyanosis. Swelling of the neck veins. The borders of the heart are dilated, the left border reaches lin. axillaris anterior. Weakened heart sounds, on the apex of the heart and on the Botkin point there heard soft systolic murmur; the heart rate is 90 per 1 min. The liver + 3 cm, the tibia are moderately swollen. On the echocardiogram there is a significant expansion of the cavities of the heart. 1. Put a preliminary diagnosis. 2. What are the signs of right type heart failure? 3. What treatment tactics should be in this case? 4. Write a prescription for Digoxin.

10. In a child of 16 during the prophylactic examination, on the ECG there have been revealed left ventricular hypertrophy. During the questioning - complaints of periodic squeezing pain in the heart, sudden attacks of weakness and dizziness. On the X-ray – bulging of the left ventricle, absence of the heart waist. 1. Put a preliminary diagnosis. 2. Make up the investigation plan. 3. What treatment tactics should be in this case? 4. Write a prescription for enalapril.

11. In a 3-year-old child who suffers from dilated cardiomyopathy, there is observed periodic anxiety, refusal from food. During active games, shortness of breath, typical asthma attack, cough, foam on the lips, cyanosis appeared. At the physical examination: expressed skin paleness, tachypnea, expanded borders of the heart, tachycardia, pulse inappropriately weak, heart sounds are weakened, and auscultated third tone and gallop rhythm. 1. What condition has developed in the child? 2. What complications may be in the patient? 3. What treatment tactics should be in this case? 4. Write a prescription for panangin.

12. In a boy of 5 years suffering from diabetes, there are complaints of the appearance of shortness of breath, feeling of lack of air during physical exertion. General blood test is in norm. On the X-ray examination - the heart is enlarged, globular. ECG revealed an abnormal Q wave, atrioventricular block 1st degree, a splitting R wave. 1. Put a preliminary diagnosis. 2. What research should be undertaken for the final diagnosis and what typical deteriorations could be revealed? 3. What treatment tactics should be in this case? 4. Write a prescription for Digoxin (sustaining dose).

13. A mother of a 4-month infant complains of the child’s refusal from food, appearance of anxiety, dyspnea, cyanosis during feeding. These symptoms are increasing. The child in the previous 4 months has not been ill. At the physical examination: the heart borders are dilated, pulse is rapid, weak, weakened heart sounds, auscultated gallop rhythm, systolic murmur on the apex of the heart, moist rales over the lungs. ECG - sinus tachycardia, signs of left ventricular overload. On the X-ray - the heart is enlarged, has the shape of a ball. 1. Put a preliminary diagnosis. 2. What research should be undertaken for final diagnosis and what typical deteriorations could be revealed? 3. What treatment tactics in this case? 4. Write a prescription for Riboxin.

14. A girl of 1 year, suffering from restrictive cardiomyopathy, was delivered by the ambulances to the hospital with the phenomena of breathing difficulty. At the examination, there were revealed diffuse cyanosis, sweating, bulging of neck veins, enlargement of the right border of the heart, tachycardia, pulse of weak filling, weakened heart sounds, accent of 2nd tone on the pulmonary artery, the liver emerges from under the edge of the costal arch by 5 cm, lower extremities edema, BP 80/40 mmHg. 1. What condition has developed in the child? 2. What diseases should be differentiated by the restrictive cardiomyopathy? 3. What treatment tactics should be in this case? 4. Write a prescription for Cocarboxilasa.

15. A 6-years-old boy complaints of cough and shortness of breath during a very slight exercise (walking). Symptoms are gradually increasing. Previously, has not been ill. Obj: the skin is pale, with acrocyanosis. Bulging neck veins. The borders of the heart are extended to the right. Weakened heart sounds; on the point of Botkin - systolic murmur; tachycardia, the 2nd tone emphasis is on pulmonary artery. The liver + 3 cm; swollen legs. On the electrocardiogram - a high pointed P-wave, an increased amplitude of R-wave, a decrease of the ST segment and the T-wave amplitude in the third abduction, aVR and right chest. 1. Which state has the child developed? 2. What treatment tactics should be in this case? 3. What drugs should be used with caution? 4. Write a prescription for prednisolone (injection solution).

B. The tests for self-control 1. A girl of 5 year. Two weeks ago she had acute tonsillitis. Objectively: heart rate is 100 beats per minute. How to estimate this data? A. tachycardia B. bradycardia C. heart rate is in norm D. respiratory arrhythmia E. paroxysmal tachycardia

2. A child of 10 year has manifestation of acute infectious-allergic myocarditis. Which of the given below drugs is necessary for treatment: A. adrenalin B. calcium chloride C. digitalis glycosides D. antiarrhythmic drugs E. glucocorticoides

3. A girl of 5 year complains of cephalalgia, flaccidity, dyspnea, palpitation. Two weeks ago she had gastroenteritis. Objectively: the condition of a child is poor. There are flaccidity, paleness, tachycardia to 120 in a minute, gallop rhythm, heart sounds are indistinct, murmurs are absent, moist rales in lungs, the liver is +2 sm. The peripheral pulse is weak, heart size enlargement. The electrocardiogram shows the change of ST segment and T wave, arrhythmia. What disease does it reasonable to suppose uppermost? A. Non-rheumatic carditis B. Infectious endocarditis C. Rheumatism D. Acute pneumonia E. Hypertrophic cardiomyopathy

4. A child of 8 year complains of the dyspnea, abdominal pain. It is known by anamnesis that for 2 weeks a child had the acute respiratory viral infection. The condition of child is moderately severe. The pulse is 200 per minute, "thread" one. There are swelling and pulsation of neck veins. Blood pressure is 60/40 mm Hg. Heart borders are extended into all directions. Heart sounds are diminished. The liver is enlarged, painful. Make a diagnosis: A. Exsudative pericarditis B. Septic endomyocarditis C. Endomyocardial fibroelastosis D. Acute non-rheumatic carditis with the affection of the conduction heart system. Paroxysmal tachycardia E. Rheumatism, myocarditis, acute course

5. Which of the given below preparations is does not use for treatment of non-rheumatic carditis: A. Prednisolone B. Indometacin C. Salicylates D. Quinidine E. Digoxin

6. A child came to permanent establishment with the acute infectiously-allergic myocarditis. On 2nd day of hospital stay in permanent establishment the attack of supraventricular paroxysmal tachycardia developed at a child. What medicine is the preparation of choice for the removal of attack? A. Finoptinum B. Noradrenalin C. Digitoxin D. Morphine E. Quinidine

7. A boy of 12 year has the acute respiratory viral infection for 5 days. During the examination the district paediatrician detected an arrhythmia, at auscultation there is the weakening of the first heart sound on the apex, short localized systolic murmur on the 5th point. On the electrocardiogram there are polytopic extrasystole, lowering of T wave amplitude. Make a diagnosis. A. Non-rheumatic carditis B. Rheumatic carditis C. Vegetal-vascular dystonia D. Bacterial endocarditis E. Secondary cardiopathy

8. A child of 12 years is diagnosed with an non-rheumatic carditis, acute, moderately severe, with the signs of heart failure of first degree in 7 days after the acute respiratory viral infection. What nosotropic mechanism underlay’s in the disease? A. Infectiously-allergic one B. Infectious one C. Allergic one D. Autoimmune one Е. Toxic one

9. 10-year-old girl after acute respiratory viral infection complaints of cardiodynia and dyspnea оп exercise. At the examination: the skin is pale. Left cardiac border is displaced to the middle clavicular line, weakening of heart sounds, inferior systolicus the арех. Heart rate is 124 per minute, blood pressure is 90/60 mm Hg. Blood: anemia, moderate leukocytosis, eosinophilia, proteins of acute phase. ECG: sinus tachycardia, disorders of repolarization processes, displacement of ST interval below isoline. Described symptoms are characteristic for: А. Non-rheumatic carditis В. Pancarditis С. Septic endocarditis D. Fibroelastosis Е. Rheumatic carditis

10. Hypertrophic cardiomyopathy is suspected in a child of 5 years. Which of the given below methods is most helpful to confirm the diagnosis: A. electrocardiogram B. echocardiogram S. Х-ray examination of the chest D. 24 hour Holter monitoring E. measuring of blood pressure

11. A boy of 12 year, after a quick walk, has developed an increased respiration rate, a sense of air deficiency, a typical asthma attack and foam on the lips have appeared. Moist rales are in the lungs. The most probable cause of the child”s state aggravation is related to: A. Acute vascular insufficiency B. total-type heart failure C. left-type heart failure D. Acute respiratory failure E. right-type heart failure

12. A girl of 12 year complaints of intermittent pain in the heart. The causes of the heartache can be: A. Psychoemotional disorders B. Cardiomyopathies C. Diseases of the respiratory system D. Pathology of the spine E. All listed

13. A girl of 7 year is in the hospital with congestive heart failure and is receiving digoxin. On the fifth day of treatment, vomiting, nausea, anorexia, headache, disturbance of color vision, liquid excrement arouse. What is the most probable cause of these complications? A. Acute renal failure B. Aggravation of underlying disease C. Digitalis toxicity D. Acute meningoencephalitis E. Infectious enterocolitis

14. Which drug is the drug of choice for treating restrictive cardiomyopathy in the earliest stage? A. Digoxin B. Furosemid C. Propranolol D. Prednisolon E. Calcium

15. A boy of 6 months was hospitalized with acute pneumonia. The chest X-ray revealed a spherical form of the heart. The Echocardiogram Data: dilatation of heart cavities, especially of the left ventricle, reduction of myocardial contractility. It is known that the child’s grandmother had cardiac disease. What disease can be suspected? A. Pericarditis B. Tetralogy of Fallot C. Myocarditis D. Pulmonary heart E. Dilated cardiomyopathy

16. In a 15-year guy, left ventricular hypertrophy was revealed on the electrocardiogram. Complaints of periodic squeezing pain in the heart were revealed. On the radiograph there is left ventricular hypertrophy. On echocardiogram there is asymmetric hypertrophy of the ventricular septum. Most likely the hypertrophy was caused by: A. Physical activity B. Mitral valve insufficiency C. Abramov - Fiedler myocarditis D. Increased blood pressure E. Hypertrophic cardiomyopathy

17. A child with Heart failure of IIA stage is receiving digoxin. There have appeared increases of bradycardia, nausea, vomiting, dizziness, sleep disturbance. On the ECG: extrasystoles, PQ – 0.18 sec. What is the possible cause of this condition? A. Acute intestinal infection B. Pulmonary edema C. 1st degree Atrioventricular block D. Overdose or intolerance of digitalis glycoside E.

18. Which medicines are contraindicated to children with hypertrophic cardiomyopathy: A. Digitalis glycosides B. Antagonists of calcium C. β-blockers D. Vitamins E. Antibiotics

19. A boy of 14 years complains of shortness of breath while walking, edema of feet. The disease has been developing gradually. Objectively: acrocyanosis. The borders of the heart are dilated; the left border reaches lin. axillaris anterior. Heart sounds are weak, soft systolic murmur is on the apex and point of Botkin, the heart rate is 94 /min, BP 120/80 mm Hg. Liver + 3 cm, the tibia are moderately swollen. On the echocardiogram there is a significant expansion of the cavities of the heart. What is the most probable diagnosis? A. Mitral heart disease B. Thonsilogenic Myocardiopathy C. Infectious-allergic myocarditis D. Dilated cardiomyopathy E. Congenital heart disease

Literature Basic: 1. Behrman Richard E., Robert M. Kliegman, Hal B. Jenson, eds. Nelson Texbook of Paediatrics. Philadelfia: WB Saunders, 18th edition - 2012. – 994 р. – Р.638. 2. Buryak V.N., Makhmutov R.F., Poshehonova J.V. Children`s diseases. – Donetsk: Nord- Press, 2010. – 328 p. 3. Pediatry. Guidance aid / Ed. by O. Tiazhka – Kyiv: Medicine, 2007.

Additional 1. Derek S. Wheeler, Hector R. Wong and Thomas P. Shanley Cardiovascular Pediatric Critical Illness and Injury, 2nd ed. 2009. 2. Illustrated Textbook of Paediatrics/ Ed.: T. Lissauer, G. Clayden.- Edinburg: Mosby, 2006.- 410 pp. 3. Forfar & Arneil’s Textbook of Pediatrics/ Ed.: N.Mc Intosh, P.J. Helms, R.L. Smyth, S. Logan.- Edinburg: Churchill Livingstone, 2008. - 1687 pp.

The methodical instructions are compiled by Bubyr L.M.

Methodical instructions were discussed and approved at the meeting of department of Pediatrics №2 protocol № ___ of ______20____.

Additions and changes were made “____” ______20____ Additions and changes were made “____” ______20____ Additions and changes were made “____” ______20____ Additions and changes were made “____” ______20____ Additions and changes were made “____” ______20____ MINISTRY OF HEALTH OF UKRAINE UKRAINIAN MEDICAL STOMATOLOGICAL ACADEMY DEPARTMENT OF PEDIATRICS №2

Approved at the meeting of department of Pediatrics №2 Minutes № 21 " 05 " 06 2020 Head of the department MD, Prof. Kryuchko T.O. ______

METHODICAL INSTRUCTIONS FOR INDEPENDENT WORK OF STUDENTS DURING THE TRAINING FOR THE PRACTICAL (SEMINARS) CLASS

Academic discipline Pediatrics Моdule № 1 Content mоdule № 2 The topic of the studies Disorder of the heart rhythm and conduction in children. Course (year) 4 Faculty Medical

Poltava 2020 1. Actuality of topic: Arrhythmia in children - is the most common pathology of pediatric cardiology. Practically there is a disease in which it had not been observed cardiac arrhythmia. In recent years there has been increase in the prevalence of arrhythmias in children, a variety of forms, sometimes the lack of effective treatment in connection with the complexity of diagnosis. Period of maximum risk of arrhythmias in children are: the neonatal period, age 4-5 years, 7-8 years, 12-13 years. According to this, it is useful in the regular clinical examination should be mandatory electrocardiographic screening in children these age groups. And if there is even minimal cardiogenic complaints, in addition to the standard survey techniques, you must assign Holter monitoring, medication stress tests, CIG, genealogical analysis of risk factors. In addition, it should be remembered that, unlike adults, children dysrhythmia is often asymptomatic and often being the child does not suffer for a long time, which greatly complicates early diagnosis of this pathology and may not accurately set the duration of the existence of arrhythmias and age of the child before the onset of illness. And in the absence of timely and adequate therapy for 4-6 years, most arrhythmias are progressing at the same time formed persistent and irreversible myocardial dysfunction requiring surgical treatment. And more than 85% of children can be cured by drug therapy when treated early. You must always remember that there is a close relationship of arrhythmias with sudden cardiac death rate is among children and young people is fairly high (0.6% of deaths in children aged 3 to 13 years, 2.3% for deaths under 22 years) . In some cases, such as the syndrome of extended interval QT, ignorance of doctors and parents about the existence of fibrillation leads to tragic consequences: the first and only in the lives of syncopal attack may result in sudden death of a child. In addition to independent significance, cardiac arrhythmias may complicate the course of other diseases cardiogenic and noncardiogenic nature, taking the character of the leading symptoms. It is therefore important to know the mechanisms of arrhythmias, clinical manifestations, diagnosis and treatment of them in order to prevent a heavy flow and the development of complications.

2. The specific aims: − To define etiologic and nosotropic violations of cardiac rhythm and conductivity in children. − To classify and analyse the clinical picture of violations of cardiac rhythm and conductivity in children. − To work out a plan of inspection and analyse information of laboratory and instrumental examinations at the typical flowing of violations of cardiac rhythm and conductivity in children. − To demonstrate a domain principles of treatment, rehabilitation and prophylaxis of violation of cardiac rhythm and conductivity in children. − To propose a preliminary diagnosis at violations of cardiac rhythm and conductivity in children. − To make the prognosis for life at violations of cardiac rhythm and conductivity in children. − To demonstrate a domain morally deontological principles of medical worker and principles of professional deference to the rank in child's cardiology.

3. Basic knowledge, skills, habits necessary for studing of the topic (inter-disciplinary integration) № The names of the The acquired skills preceding disciplines 1. Normal anatomy To know the anatomic features of the cardiovascular system of children 2. Normal physiology To own knowledge about physiology processes which take a place in the cardiovascular system of children. 3. Physiopathology To identify physiopathology processes which arise up in the cardiovascular system on the basis of found out symptoms.

4. Care of patients and To demonstrate a domain skills of care of children with pathology nurse practice of the cardiovascular system and grant pre-medical Medicare. 5. Propedeuvtics of To own skills of collection of anamnesis, clinical inspection of pediatrics child, by knowledge about the basic clinical symptoms of children with pathology of the cardiovascular system, to know about the laboratory and instrumental methods of inspection. 6. Social medicine and To apply knowledge about the structure of grant of medical & organization of sanitary help to child's population for the proper use of resources of health protection the system of health protection in the plan of treatment and prophylaxis of origin of diseases of the cardiovascular system. 7. Pharmacology To own knowledge about the basic groups of medicinal facilities which are used in treatment of diseases of the cardiovascular system of children

4. The tasks for independent work during the training for the studies. 4.1. The list of basic terms, parameters, characteristics, which the student should master while training for the studies: Term Definitions 1. Arrhythmias - Any heart rate that differs from normal frequency, regularity, violation of the pulse sequence of activation and ventricular fibrillation. 2. Extrasystoles Premature excitation and reduction of myocardium, which occurs on the background of sinus rhythm. 3. Type of cardiac rhythm -Violation of the occurrence of the impulse; - Violation of the conduction of the impulse; - Combination arrhythmias 4. The main instrumental methods of ECG, rytmohramm, standard of physical diagnosing arrhythmias activity, captopril test, Holter ECG monitoring

5. Main antiarrhythmic drugs novokayinamid, difenin, lidocaine, verapamil, izoptin, etmozin, etatsyzyn, kordaron, digoxin, ATP, propranolol

4.2. The theoretical questions to the studies: Theoretical issues on which the target might implement activities arising from the graph logical structure of the subject content: 1. Leading clinical symptoms and syndromes in beats, paroxysmal tachycardia, atrial fibrillation, complete AV block. 2. The clinical variants of paroxysmal tachycardia and atrial fibrillation in children. 3. Necessary tools for the study beats, paroxysmal tachycardia, atrial fibrillation, complete AV block. 4. Differential diagnosis of extrasystole, paroxysmal tachycardia, atrial fibrillation and complete AV-blockade. 5. Tactics of the patient with extrasystoles, atrial tachycardia, atrial fibrillation, complete AV block in children. 6. Provision of emergency assistance in paroxysmal tachycardia, atrial fibrillation, Morgagni- Adams-Stokes syndrome in children. 7. Prevention of cardiac arrhythmias and conduction in children.

4.3. The practical works (tasks) which are done at the studies: 1. Work with test tasks. 2. Work of students in chambers near the bed of children of patients with disorder of the heart rhythm and conduction in children. 3. Interpretation of additional methods of inspection and differential diagnostics of diseases. 4. Clinical analysis of a typical case. 5. Solving of the situational tasks.

5. The contents of the topic:

Classification of arrhythmias (NA Belokon, 1987)

I. Violations pulse formation. A. Nomotopic dysrhythmia (violation of momentum in the formation of sinus node) 1) sinus arrhythmia 2) sinus bradycardia 3) synus tachycardia 4) migration rate driver B. Heterotopic (ectopic) dysrhythmia - impulses arise outside the sinus node. 1) premature: - supraventricular - ventricular; 2) paroxysmal tachycardia: - supraventricular, - ventricular; 3) Nonparoxysmal tachycardia: - Atrial - Of atrioventricular connection - Ventricular; 4) flutter and auricular fibrillation (atrial fibrillation); 5) flutter and ventricular fibrillation. II. Conduction - Sinoauricular block - Atrial block - Atrioventricular block - Ventricular block III. Combined Arrhythmia - Weak sinus syndrome - Atrioventricular dissociation - Preexcitation syndrome.

DIAGNOSIS AND TREATMENT CONDUCTION DISTURBANCE

Delayed first-degree Incomplete AV-block I degree DIAGNOSTIC CRITERIA Clinical: - Course of clinically asymptomatic Paraclinical: - EKG: PQ interval prolongation registered more than 0.18 seconds (in infants over 0.15 sec). To clarify the nature and origin of the siege show klinoortoprob. If the blockage persists after klinoortoprob, applying tests with atropine - Holter daily monitoring of heart rate . TREATMENT - When the functional blockade regime without any restrictions. When the organic - the regime depends on the severity of the underlying disease of the heart and the presence of heart failure. - Treatment of the underlying disease of the heart. - Detection at Holter monitoring during night AV-block ΙΙ degree in healthy children does not require further examination or specific therapy. - In the presence of a functional AV-block ΙΙ degree on the background of clinically manifested vagotonia can appoint bellaspon or bellataminal. - If there is autonomic dysfunction with a predominance vagotonia - hydrotherapy: cool and douches, bath.

Delayed second-degree - Incomplete AV-block II degree and type with periods Samoilova-Venkenbaha (Mobitts type I); - Incomplete AV-block II degree type II (type II Mobitts). DIAGNOSTIC CRITERIA Clinical: - Often asymptomatic, can be observed bradycardia and clinical symptoms vagotonia. Organic II degree AV block is accompanied by symptoms of the underlying disease of the heart. Paraclinical: - ECG-diagnostics: AV block II degree (Type Mobitts I) - recorded a consistent progressive lengthening of the interval PQ, that ends in total prolapse of the complex QRS, and then recorded a long pause. wave P and QRS on the ECG is not deformed. AV block II degree (Type Mobitts II) - is characterized by ECG abrupt cessation of impulse conduction in the ventricles and the fallout of QRS complex with a multiplicity of 2, 3, 4 or more cardiac and pause after wave P. The number of P wave more than complexes QRS. PQ intervals have the same duration. - Holter daily monitoring of heart rate. TREATMENT - When the functional blockade regime without any restrictions. When the organic - the regime depends on the severity of the underlying disease of the heart and the presence of heart failure. - Treatment of the underlying disease of the heart. - Appointment of cardiometabolites and neurometabolites on the relevant testimony. - In a significant bradycardia - sympathomimetic (epinephrine Izadrin) symptomatically.

Delayed full Third degree atrioventricular block (complete AV-block ). DIAGNOSTIC CRITERIA Clinical: - Bradycardia, - Flapping ΙΙ tone at the top of the heart, - Systolic murmur, diastolic noise at the top, - Possible attacks Morgagni-Adams-Stokes syndrome. Paraclinical: - ECG-diagnostics: With full AV-blockade atrial reduced at their own pace, and the ventricles in a slower pace. The ECG P wave complexes not associated with QRS. Intervals RR - regular intervals PP - different. QRS complexes may be normal and distorted with the extension of more than 0.11 seconds (ventricular pacemaker is located in one of the legs of the beam His) - Pharmacological assay with atropine, - Daily monitoring of cardiac rhythm Halter. TREATMENT - In the absence of clinical symptoms heart disease treatment sparing with limited physical activities (physical exercise, sports). When the organic AV-blockade III degree regime depending on the severity of the underlying disease of the heart and the presence of heart failure. - Treatment of the underlying disease of the heart. - Cardiometabolites (riboflavin, calcium pangamat, lipoic acid, etc.). - Sympathomimetics (epinephrine, Izadrin used symptomatically with significant bradycardia and the threat of syncope). - Surgical treatment. In the treatment of complete AV-blockade in children of different etiologies effective method is temporary or permanent pacing. Temporary pacing can be used for emergency treatment of acquired complete AV-blockade. Indications for implantation of physiological pacing in congenital complete AV-blockade: bradycardia less than 40 beats per minute, and syncopal syncope (Morgagni-Adams-Stokes syndrome ), circulatory failure, limitation of physical activity.

DIAGNOSIS AND TREATMENT OF PULSE FORMATION VIOLATIONS

Atrial extrasystole DIAGNOSTIC CRITERIA Clinical: - Auscultative data - Complaints are usually not available, may be "failures" in the heart. Paraclinical: - ECG: premature appearance of all major elements of the ECG with P wave, the normal sequence of spikes P, Q, R, S, T the normal form of the complex QRS; tine P extrasystoles have different morphology and polarity, compared with the P wave of sinus rate, the interval PQ shortened or lengthened (rare). - Grouped extrasystoles may progress to atrial paroxysmal tachycardia and fibrillation. In such cases, the most informative for the diagnosis is EEFI and daily Holter monitoring of cardiac rhythm. TREATMENT - Identifying with Holter monitoring unit extrasystoles in healthy children does not require further examination or specific of antiarrhythmic therapy. - Presence of frequent extrasystoles (more than 250-300 per hour) requires the dispensary observation and monitoring the development of arrhythmogenic cardiomyopathy. - Mode without restrictions. Normal diet. Sports resolved after the treatment of the disease and the elimination of beats. - Treatment of the underlying disease (the autonomic dysfunction, organic heart disease, etc.), readjustment of foci of chronic infection. - Antiarrhythmic drugs are appointed only if the group or politopnyh extrasystoles, leading to hemodynamic disturbances, the threat of paroxysmal tachycardia or atrial fibrillation: verapamil, amiodarone, etc., salts of potassium (panangin, asparcam).

Atrioventricular extrasystole DIAGNOSTIC CRITERIA Clinical: - Auscultative data - Complaints are usually not available, may be "failures" in the heart. Paraclinical: - EKG: There are three variants of the AB-arrythmia with: 1) the simultaneous excitation of the atria and ventricles, 2) with preliminary excitation of the ventricles, and 3) with preliminary excitation of the ventricles and complete blockade of retrograde (stem extrasystoles from the bundle of His). For all AV-beats characteristic of the normal form of the ventricular complex. The first version of the P wave is not available; ventricular complexes are not changed or slightly deformed by stratification P; compensatory pauses incomplete. In the second version of the recorded normal or aberrant ventricular complex, after an interval of 0,06-0,10 - negative prong P (derivations II, III, avF) and positive P in lead avR; full compensatory pause. In the third option after unmodified or aberrant complex ventricular recorded positive prong P sinus origin; compensatory pause is complete. - The most informative for diagnosis is EEFI and daily holter monitoring. TREATMENT - Detection at Holter monitoring unit atrioventricular extrasystole in healthy children does not require further examination or specific antiarrhythmic therapy. - Mode without restrictions. Normal diet. Sports resolved after the treatment of the disease and the elimination of beats. - Treatment of the underlying disease (the autonomic dysfunction, organic heart disease, etc.), readjustment of foci of chronic infection. - Antiarrhythmic drugs are appointed only if the group or polytopic extrasystoles, leading to hemodynamic disturbances, the threat of paroxysmal atrial fibrillation or tachycardia: verapamil, amiodarone, and others, potassium salt (panangin, asparcam).

Ventricular extrasystole The causes of ventricular arrhythmias may be myocarditis, cardiomyopathy dysmetabolic, the presence of additional conduction pathways, scoliosis of the thoracic spine. Single ventricular premature beats observed in the autonomic dysfunction. DIAGNOSTIC CRITERIA Clinical: - Auscultative data - Complaints are usually not available, may be "intermittent", heartbeat Paraclinical: - ECG: characterized by absence of P wave, the presence of deformed ventricular premature complexes increase in amplitude and duration (more than 0,11 c); ST segment is absent or reduced; T waves high, discordant to the primary wave extrasystoles; compensatory pause is complete. - The most informative for diagnosis is EEFI and daily Holter monitoring of cardiac rhythm. TREATMENT - Detection at Holter monitoring of single ventricular extrasystoles in healthy children does not require further examination or specific antiarrhythmic therapy. - Mode without restrictions. Normal diet. Sports resolved after the treatment of the disease and the elimination of beats. - Antiarrhythmic drugs are misused in the presence of group or polytopic extrasystoles, leading to hemodynamic disturbances, the threat of development of paroxysmal tachycardia or atrial fibrillation (Etmozin 3 mg / kg / day for 3 admission; Aymalin 2-3 mg / kg / day for 3 admission; meksiletin 2 mg / kg 3 times a day, etc.). When ventricular extrasystoles III-V-class Lown drug of choice is amiodarone. - Treatment of the underlying disease (the autonomic dysfunction, organic heart disease, etc.), readjustment of foci of chronic infection. Clinical supervision: when frequent, malignant and sympathoadrenal dependent EC are entirely exempted from physical education. With rare and vagozavisimyh ES Showing special group gymnastics. Control examination was performed in 1,5 - 2 months after starting treatment, the use of antiarrhythmic drugs - monitoring the effectiveness of 2-3 weeks. Hospitalization of 1-2 times a year to complete the survey. With the disappearance of complaints, the absence of extrasystoles in the ECG, while HM - the frequency of ES does not exceed 3 per hour - the patients are removed from the dispensary.

PAROXYSMAL TACHYCARDIA Supraventricular tachycardia The cause could be the background of neuroses in the residual-organic lesion of the CNS, hypertension-hydrocephalic syndrome, vegetative-vascular dysfunction with sympathetic- adrenal crises syndrome WPW, organic heart disease (myocarditis, cardiomyopathy, heart defects). DIAGNOSTIC CRITERIA Clinical: - Is characterized by an increase in heart rate to 140-200 beats per minute, a sudden onset and sudden cessation of attack. Duration of attack from several seconds to several hours and even days. An increase in heart rate over 220-250 per minute, and syndrome small cardiac output. Paraclinical: - EKG: atrial in the form of paroxysmal tachycardia recorded a number of consecutive atrial premature beats (at least 4-6 with a frequency of more than 160 per minute). P wave varied forms of (+, -) or not determined. QRS complex is not changed. Can stratify transient incomplete AV block I-II degree. The most informative for diagnosis is transesophageal electrophysiological study of the heart and leading daily HM. TREATMENT: Child lie in a horizontal position and provide access to fresh air. Perform a sequence of reflex actions, increases the tone of the vagus nerve: in children older than 3-4 years old: Sample Valsavy (straining at the nose closed for 10 seconds), carotid sinus massage in carotid arteries during the first 5-10 seconds on the right, in the absence of effect - on the left, additional techniques used in children older than 7 years old: Pressing the spatula on the tongue root, slow deep swallowing, sponging with cold water. - Sedatives: Korvalol, valokordin, valerian (1 drop per year of life), Asparcam (panangin) 1 / 3 - 1 Table. depending on age. - If there is no effect on the reflex techniques and sedatives used antiarrhythmic drugs in the following sequence: - Verapamil 0,25% solution by slow intravenous injection (no dissolution) under the control of blood pressure and heart rate in a dose: up to 1 year 0,4-0,8 ml 0,8-1,2 ml of 1-5 years, 6-10 years 1,2-1,5 ml 1,5-2,0 ml of 11-15 years. Verapamil is contraindicated in the form of supraventricular PF with aberrant ventricular complexes in children 1 year of life (development of severe ), the syndrome WPW. The drug should not be administered simultaneously with chinidin and beta-blockers. - ATP 1% solution intravenously at a dose of fast jet 0,5-1,0 ml preschoolers and 1,0 ml - children of school age; - Aymalin (giluritmal) 2,5% by slow intravenous injection at 10,0-20,0 ml 0.9% NaCl solution at a dose of 1 mg / kg; - 0,025% digoxin intramuscularly or intravenously. Loading dose 0,03-0,05 mg / kg. The rate of saturation - 3 days. Maintenance dose - 1/5-1/6 of the loading dose. Digoxin is contraindicated in the form of supraventricular PF with aberrant ventricular complexes. - In the absence of the effect of antiarrhythmic therapy - transfer the patient in the ICU or cardiorheumatological Center. Amiodarone 5% solution intravenously very slowly at 10,0-20,0 ml 5% glucose solution at a dose of 5 mg / kg. In the absence of effect - consultation cardiologists on the need for transesophageal pacing and countershock

Ventricular paroxysmal tachycardia ETIOLOGY: organic myocardial damage: carditis, cardiomyopathy, heart diseases, post myocarditis cardiosclerosis; intoxication drugs digitalis, quinidine, hypo-and hyperkalemia syndrome elongated interval QT. Very rarely develops in the vascular dysfunction, thyrotoxicosis, psychophysical overload. Chronic (continuous-recurrent) ventricular paroxysmal tachycardia causes severe hemodynamic changes and is a condition that poses a threat to life (the transition to ventricular fibrillation) and the progression of heart failure. DIAGNOSTIC CRITERIA Clinical criteria: an increase in heart rate to 120-250 beats per minute, sudden onset and sudden end of attack. With an increase in heart rate more than 120-140 beats per minute, may develop cardiac output syndrome small. Continuous-recurrent form is characterized by frequent relapses and chronic course. Paraclinical: - ECG: record "shots" of successive ventricular extrasystoles (more than 5) with short periods of sinus rhythm. QRS complexes wide (over 0.1 sec), rough, discordant T wave to the core set of teeth QRS. P wave recognize rarely due to the lamination on the other elements of the ECG. Ventricular paroxysmal tachycardia may be mono-or polymorphic. Polymorphic or chaotic ventricular paroxysmal tachycardia is a risk of ventricular fibrillation. A variant of polymorphic ventricular tachycardia is paroxysmal tachyarrhythmia type "pirouette". With the risk of sudden cardiac death: recurrent syncopal states; polymorphic form of paroxysmal tachycardia, bradycardia least 48 beats / min. at night, long QT more than 480 msec; alternation of the T wave, breaks the rhythm more than 1,5 seconds, the presence of ventricular late potentials. TREATMENT Mode sparing with limited physical exercise, get enough sleep, fresh air. During the attack mode of bed. Sports contraindicated. - A diet enriched products containing vitamins, potassium and magnesium (raisins, dried apricots, baked potatoes, dried ). Contraindicated in some strong tea, coffee, chocolate. - Cupping: Lidocaine 1% by slow intravenous injection at a dose 1-1,5 mg per kg. In the absence of effect you can repeat the administration of lidocaine in 5-10 minutes at half the dose. In the absence of effect - Aymalin (giluritmal) 2,5% solution intravenously very slowly at 10,0 -20,0 ml 0,9% NaCl solution at a dose of 1 mg / kg. In the absence of effect of amiodarone, the introduction of 5% solution intravenously very slowly at 10,0-20,0 ml 5% glucose solution at a dose of 5 mg / kg. When no effect - consultation on the need for cardiologists, transesophageal pacing or countershock. - The prevention of thromboembolism in patients with mitral heart disease, hypertrophic cardiomyopathy, thromboembolism before and after the planned electrical cardioversion in 2-3 weeks designate indirect anticoagulants (neodikumarin). - If there is an additional source of tachycardia, after the electrophysiological study used the method of radiofrequency catheter ablation. In the absence of effective conservative therapy by implantation of cardioverter-defibrillators. - Etiopathogenetic therapy is the main disease of the heart. - According to testimony neurotropic means. - Cardiac glycosides in ventricular paroxysmal tachycardia contraindicated.

FIBRILLATION AND ATRIAL FLUTTER Fibrillation (Blink) fibrillation: paroxysmal, permanent The causes are rheumatic heart disease, rheumatic heart disease, Non-rheumatic carditis, cardiomyopathy, hyperthyroidism, digitalis intoxication, overdose of diuretics, sympathomimetics, hypokalaemia syndrome, WPW, sick sinus syndrome. Atrial fibrillation is one of the most common types of arrhythmias in patients with acquired mitral valve defects. Fibrillation (flicker) is characterized by disorganized atrial heart rhythm, in which no co- ordinated reduction in atrial myocardium, the frequency of which can reach 400 or more per minute. In this case only a portion of these reductions is transmitted through the AV connection to the ventricles (up to 200-220 impulses. / Min). Other pulses are blocked. DIAGNOSTIC CRITERIA Clinical: - There are regular (rack) form of atrial fibrillation duration up to 1-2-weeks or more and paroxysmal. If you have atrial fibrillation develops rapidly syndrome small cardiac output, there is increasing manifestations of left ventricular heart failure, there is a risk of thromboembolism. Paraclinical: - EKG: record waves of different amplitude and width with a frequency of 400-600 per minute. Expressed in leads II, III, avF, V1-2. Ventricular complex, irregular, normal form, or broad and deformed at the expense of intraventricular conduction; - Daily Holter monitoring: carried out to assess heart rate variability, clarifying the mechanisms beginning and ending of arrhythmia, interval dispersion QT; - Echocardiography: a violation is determined the functional capacity of the ventricles, can be dilated heart chambers, the presence of intracardiac thrombi. Transesophageal echocardiography is the method of choice to detect intracardiac thrombus, the need for and duration of anticoagulation therapy in patients with atrial fibrillation. - If you have syncopal episodes in history, sinus node dysfunction in the ECG of patients showed EEFI. TREATMENT Docking of atrial fibrillation should be held as soon as possible, immediately after verification of cardiac arrhythmias using the ECG, the patient is hospitalized in the intensive care unit and intensive care. - Verapamil 0,25% by slow intravenous injection at 10,0-20,0 ml 5% glucose solution at a dose of 0.15 mg / kg. - Propranolol 0.1% intravenously very slowly in a dose of 0,1-0,2 mg / kg. - In an intensive care unit and intensive care in the absence of the effect of the introduction of verapamil and propranolol, in the presence of small cardiac output syndrome and progression of heart failure are appointed by cardiac glycosides. Digoxin 0,025% intravenously. Loading dose 0,03-0,05 mg / kg. The rate of saturation - 3 days. Maintenance dose - 1/5-1/6 of the loading dose. At the same time prescribe medications potassium (panangin, Asparcam). Coagulation parameters are defined and solved with the appointment of anticoagulants. - Drug of choice for further pharmacological cardioversion is amiodarone in the form of 5% solution intravenously very slowly, at a dose of 5 mg / kg in 10,0-20,0 ml 5% glucose solution. Upon receipt of effect of switching to oral administration. - With the threat of pulmonary edema, marked hemodynamic, refractory heart failure being urgent countershock. - When bradikarditicheskoy form of atrial syncope, and appears, to a permanent pacing in cardiac clinic in the heart of implanted cardiac pacemaker. - Treatment of the underlying disease. Mode sparing with limited physical exercise, get enough sleep, fresh air. During the attack - the regime of bed. Sports contraindicated. - A diet enriched with products containing vitamins, potassium and magnesium (raisins, dried apricots, baked potatoes, dried fruit). Contraindicated in some strong tea, coffee, chocolate.

Atrial flutter The causes are rheumatic heart disease, cardiomyopathy, congenital heart disease with an overload of the atria, chronic pulmonary heart, sick sinus syndrome. Atrial flutter is one of the most common types of arrhythmias in patients with acquired mitral valve defects. Atrial flutter - atrial fibrillation, in which there is rapid rhythmic excitation and reduction of the atria with a frequency of 250-400 pulses. minute. The frequency of contractions of the ventricles as a result of a functional AV-blockade is less than the frequency reduction of atrial and is registered in the ratio of 1:2, 1:3, 1:4. DIAGNOSTIC CRITERIA Clinical: - The children complain of palpitations during physical or psychological and emotional stress, false angina, headache, dizziness. Paraclinical: - EKG: record waves with a frequency of 250-300 pulses. minute. The waves are expressed in leads II, III, avF, V1-2. Isoelectric line is absent. Ventricular complexes normal, wide or deformed when a breakdown of intraventricular conduction, recorded with a frequency of 120- 150 per minute, in proportion to the waves as 1:2, 1:3 and less. - Transesophageal echocardiography is the method of choice to detect intracardiac thrombus, the need for and duration of anticoagulant therapy in patients with of atrial flutter. TREATMENT - Docking of atrial flutter be held immediately after the diagnosis by ECG. After emergency treatment the patient should be hospitalized in cardiorheumatological department for treatment of basic disease, which led to the development of atrial flutter. According to the testimony - hospitalization in the resuscitation and intensive therapy. - Verapamil 0,25% by slow intravenous injection at 10,0-20,0 ml 5% glucose solution at a dose of 0.15 mg / kg. - Propranolol 0.1% intravenously very slowly in a dose of 0,1-0,2 mg / kg. - In an intensive care unit and intensive care in the absence of the effect of the introduction of verapamil and propranolol, in the presence of small cardiac output syndrome and progression of heart failure are appointed by cardiac glycosides. Digoxin 0,025% intravenously. Loading dose 0,03-0,05 mg / kg. The rate of saturation - 3 days. Maintenance dose - 1/5-1/6 of the loading dose. At the same time prescribe medications potassium (panangin, Asparcam). - Antiarrhythmics (digoxin, amiodarone) in supporting dosages are appointed for 3-4 weeks, followed by the cancellation in the presence of a persistent effect. - If recurrent atrial flutter method of choice for the restoration of cardiac rhythm is transesophageal electrostimulation in combination with low doses of amiodarone. - Treatment of the underlying disease. Mode sparing with limited physical exercise, get enough sleep, fresh air. During the attack - the regime of bed. Sports contraindicated. - A diet enriched with products containing vitamins, potassium and magnesium (raisins, dried apricots, baked potatoes, dried fruit). Contraindicated in some strong tea, coffee, chocolate.

FIBRILLATION AND VENTRICULAR FLUTTER Fibrillation and ventricular flutter - a very severe state of cardiac activity, leading to its cessation and death. Atrial flutter is characterized by ventricular ectopic ventricular pulses with a frequency of 250-300 pulses. per minute and the lack of diastole. Fibrillation (flicker) of the ventricles is characterized by chaotic decline of individual sections of ventricular rate with more than 300 counts. per minute, leading to cessation of blood circulation. The causes are congestive heart failure, cardiomyopathy, shock, and hypoxic conditions, the terminal stage of many somatic diseases. DIAGNOSTIC CRITERIA Clinical: - Circulatory arrest, loss of consciousness, - Clinical Death Paraclinical: - ECG: ventricular flutter recorded in the sawtooth wave without differentiating normal EKG elements (teeth P, T, QRS complex are absent). Pulse frequency of 200-300 per minute. Isoelectric line is absent. When ventricular fibrillation waves more frequent (more than 300 per minute), variable shape, width and amplitude. Diastole is completely absent. Atrial flutter and ventricular fibrillation may precede AV block II-III degree, idioventricular rhythms; - The method of choice to detect intracardiac thrombi in atrial fibrillation, the definition of indications for and duration of anticoagulation therapy is transesophageal echocardiography. TREATMENT Emergency medical therapy before and during hospitalization, shows the transfer of the patient in intensive care unit. - Against the background of primary reanimation procedures (release of the respiratory tract, tracheal intubation, artificial ventilation, closed heart massage) the patient should be urgently undertaken defibrillation. Initial energy level - 2 J / kg. In the absence of effect - the energy of the next level can be increased to 4 J / kg. All of the following levels should be combined with a / in the introduction of epinephrine (0.01 mg / kg) and intervals of not less than 2-3 minutes. Maximum energy level - 360 J. - If there is no effect appointed novokainamid 10% solution intravenously at a dose of jet 1,0-3,0 ml depending on age or lidocaine 1% solution by slow intravenous injection at a dose of 1 mg / kg. After the introduction of the drug - again defibrillation. - After coping with the attack and the improvement of children given a diet enriched with products containing vitamins, potassium and magnesium. Limited sodium chloride and liquid. Mode - depending on the underlying disease. - Must be treatment of underlying disease.

Emergency care when Morgagni-Adams-Stokes - syndrome - At the pre-hospital (hospital) stage in the development of attack Morgagni-Adams-Stokes made the urgent measures. Provides free access of air to the respiratory tract the child, if possible carry oxygen. Atropine sulphate 0,1% intramuscularly at the root of language or intravenously in a dose 0,005-0,01 mg / kg or 0.05 ml / year of life. In the absence of the effect of atropine can be used aminophylline 2.4% solution intravenously at a dose of 2-4 mg / kg. - Provide urgent hospitalization of the child in intensive care. - With the development of asystole - carry out cardiopulmonary resuscitation. - To eliminate the small cardiac output syndrome with medical therapy carry a sick child, an increase in heart rate above a critical level: 0,1% atropine sulfate solution intravenously at a dose of 0,005-0,01 mg / kg, or 0.05 ml / year of life, Izadrin intravenously at a dose of 1-2 mg / (kg / min), dopamine intravenously at a dose of 5-8 mg / (kg / min). - Repeated attacks of Morgagni-Adams-Stokes - Consultation cardiologists to address the urgent surgical implantation of artificial pacemaker (pacemaker) in cardio surgery center.

6. The materials for self-control A. Sample case report Situational problem № 1 The boy is 4 days. My mother is sick with systemic lupus erythematosus. With the birth of a child's heart rate did not exceed 60 minutes. OBJECTIVE: the general state of the heavy, body temperature 35,7 ° C, listless child, during the feeding quickly gets tired of the food refuse, skin and mucous membranes are pale, cold extremities, acrocyanosis little moans, "Marble" image of the skin, muscle hypotonia. BH 36 min. Above the lungs- vesicular breath, clear tone percussion sounds. HR 48 in minutes. Borders of the heart is not enlarged. Heart sounds loud and clear. The abdomen was soft, the liver and spleen were not enlarged. Complete blood count: Hb 132 g / l, Erythr.- 3,7 T / l; CIB -0,9; Leuk. -10 G / l neutrophils: stab-9%, segm- 43% eosin-3% lymph-42%, mon-3%, ESR - 17 mm / h. A radiograph of the chest cavity - changes not revealed. On echocardiography - age dilatation of the right ventricle and atrium, patent foramen ovale, pulmonary hypertension of newborns.

Answer the following questions: 1. What is the leading clinical syndrome? 2. Provide the differential diagnose according to the main estimated clinical syndrome. 3.Put the previous clinical diagnose. 4.Create the plan of the clinical examination. 5.Create treatment strategy.

Situational problem № 2 Girl 6 months. Complaints mother to child anxiety, shortness of breath, fever, refusal to eat. OBJECTIVE: general condition of the child moderately, reflecting moderate intoxication syndrome on the background of catarrh of the upper respiratory tract and significant tachycardia. The body temperature of 38.5 C, the child is restless, listless. Skin and visible mucous membranes are pale pink, clean, slimy mouth moderately hyperemic. Pulse 210 per minute, fast, superficial, weakened. The boundaries of the relative stupidity of the heart within the age norms for auscultation tones are clear and clean. Nasal breathing is difficult because of mucous secretion. Above the lungs - clear percussion sounds, harsh breath auscultation, respiratory rate - 50 min. Abdomen palpation soft nonpathologic, liver 3 cm below the costal arch on the mid clavicular line, the edge of elastic, spleen not palpable. Feces was the morning, urine output is sufficient. Complete blood count: Hb - 122 g / l, er. - 3,9 T / l, WBC - 8,0 G / l, neutrophils: stab 2% - segmentolnuclear - 35%, eosinophils - 3%, lymphocytes - 53%, monocytes - 7%, ESR – 11 mm / h . ECG: see (HR - 260 beats per minute, the vertical position of the electrical axis of heart). Answer the following questions: 1. What is the leading clinical syndrome? 2. Provide the differential diagnose according to the main estimated clinical syndrome. 3.Put the previous clinical diagnose. 4.Create the plan of the clinical examination. 5.Create treatment strategy.

Situational problem № 3 Children 6 months. At the age of 1 month diagnosed early congenital carditis (fibroelastosis). Admitted to the hospital at the sharp deterioration. OBJECTIVE: the dire state, shortness of breath and 60 minutes in a moderately pronounced cyanosis of the skin, increases with a baby crying, slimy with a purple tinge. Auscultatory listened multiple small bubbling moist rales mostly in lower divisions. Pulse 180 per minute, filiform. Cardiac impulse is diffused. Borders of the heart significantly expanded in all directions, mostly to the left. Heart sounds relaxed, tripartite rhythm "gallop". Liver on the level of the navel. Edema of the labia majora, the lower extremities. X-ray examination WGC: megalocardia, increased lung pattern. Echocardiography: dilatation of all chambers of the heart with reduced left ventricular systolic function (EF 25%).

Answer the following questions: 1. What is the leading clinical syndrome? 2. Provide the differential diagnose according to the main estimated clinical syndrome. 3.Put the previous clinical diagnose. 4.Create the plan of the clinical examination. 5.Create treatment strategy.

Situational problem № 4 Girl of 6 years. Is treated for acute glomerulonephritis. OBJECTIVE: body temperature of 37,0 ° C, respiratory rate 24 per minute, pulse rate 104 per minute, blood pressure 145/105 mm Hg Status girls heavy, moderate edematous syndrome. Heart sounds are muffled, rhythmic. Percussion over the light - bright lung sound auscultation - vesicular breathing. The abdomen was soft, there is tenderness at percussion in the lumbar area on both sides. No urine for 12 hours. Complete blood count: Hb - 110 g / l, erythrocyte - 3,5 T / l, WBC - 8,2 G / l, eosinophils -4%, stab neutrophils - 5%, segmentolnuclear - 42 %, lymphocytes - 40%, monocyte - 9%, ESR - 19 mm / h. Urinalysis: muddy, colors "meat slops", protein 1.2 g / l, glucose was no found, erythrocytes cover all the field of view, cylinders granular, white blood cells 8-10 in the field of view. Serum potassium 9.0 mmol / l. Diagnosis oligoanurial stage of acute renal failure. During the transfer to the intensive care the child lost consciousness, marked mydriasis, absent peripheral pulse, surface bradipnoe, EKG - see Fig. Answer the following questions: 1. What is the leading clinical syndrome? 2. Provide the differential diagnose according to the main estimated clinical syndrome. 3.Put the previous clinical diagnose. 4.Create the plan of the clinical examination. 5.Create treatment strategy.

Situational problem № 5 The boy of 5 years with a planned survey of kindergarten was revealed a marked bradycardia, about which the child was sent to hospital for examination. Complaints on admission to rapid fatigue, feeling of general weakness, sweating with exertion. The general condition of the child is satisfactory. The boy asthenic physique, low fatness. Skin and mucous membranes of pure, pale-pink color. Peripheral nodes were not enlarged. In lung auscultation vesicular breath. Percussion limits of relative cardiac dullness: right - 1 cm outwards from the right edge of the sternum, the upper - the second intercostal space, left - 1 cm out from the left mid-clavicular line. Auscultation at the top of the first tone is muted, the emphasis ΙΙ tone of the pulmonary artery systolic murmur at the top of a functional nature. HR - 44 per minute, BP - 90/60 mm.Hg. In general analysis of blood, immunebiochemical parameters revealed no abnormalities. ECG attached.

Answer the following questions: 1. What is the leading clinical syndrome? 2. Provide the differential diagnose according to the main estimated clinical syndrome. 3.Put the previous clinical diagnose. 4.Create the plan of the clinical examination. 5.Create treatment strategy.

Situational problem № 6 Female 12 years old, was admitted to hospital complaining of pain in the heart, headaches, seizure brief loss of consciousness, weakness. From history we know that is sick for 2 years, but attacks of unconsciousness occurs first. OBJECTIVE: pale skin, moderate acrocyanosis. Percussion border of the heart dilated left at 1,5 cm out from the left mid-clavicular line.. Auscultation - heart rhythm is wrong, bradyarrhythmia, auscultated systolic murmur at apex and at the point Botkin- Urbana. The ECG - sinus rhythm, bradycardia, a gradual increase in the range P-Q, followed by precipitation of the complex QRS, violation of myocardium repolarization. Answer the following questions: 1. What is the leading clinical syndrome? 2. Provide the differential diagnose according to the main estimated clinical syndrome. 3.Put the previous clinical diagnose. 4.Create the plan of the clinical examination. 5.Create treatment strategy.

Situational problem №7 Female 8 years old, treated in the cardiorheumatological department about non-rheumatic carditis. On day 5 of illness, the child developed an attack of tachycardia to 180 beats per minute, which was accompanied by severe weakness, sweating, anxiety, lower blood pressure to 80/40 mm Hg. ECG attached

Answer the following questions: 1. What is the leading clinical syndrome? 2. Provide the differential diagnose according to the main estimated clinical syndrome. 3.Put the previous clinical diagnose. 4.Create the plan of the clinical examination. 5.Create treatment strategy.

Situational problem №8 Boy 11 years. Appeals for entrance to the heartbeat, dizziness, pain in the heart feeling of general weakness. From history we know that a child is at dispensary cardiologist about the phenomenon of WPW, above complaint first arose. OBJECTIVE: skin and mucous membranes of pure, pale- pink color. Peripheral nodes were not enlarged. In lung auscultation vesicular breath. Percussion boundaries relative cardiac dullness is not extended. Auscultation heart sounds are regular, systolic murmur at the apex of a functional nature. Pulse 210 per minute, fast, superficial, weakened. ECG attached:

Answer the following questions: 1. What is the leading clinical syndrome? 2. Provide the differential diagnose according to the main estimated clinical syndrome. 3.Put the previous clinical diagnose. 4.Create the plan of the clinical examination. 5.Create treatment strategy.

Situational problem №9 Boy 2 years. Located under medical supervision at the congenital heart defects: ventricular septal defect after surgery (plastic interventricular septal defect). OBJECTIVE: lag in physical development, paleness of the skin. On the chest scar after the heart operation. Percussion of the heart borders extended to the left to 1,5 cm auscultation heart sounds more relaxed, rhythmic, with a frequency of 52 per min. Arterial pressure was 70/40 mmHg Liver 2 cm emerges from under the costal arch. The lower extremities noted pasty. At the next inspection recording ECG boy lost consciousness. ECG attached

Answer the following questions: 1. What is the leading clinical syndrome? 2. Provide the differential diagnose according to the main estimated clinical syndrome. 3.Put the previous clinical diagnose. 4.Create the plan of the clinical examination. 5.Create treatment strategy.

Situational problem №10 A boy of 16 years, treated in cardiorheumatological department regarding dilated cardiomyopathy. Complaints of shortness of breath with little exertion, general weakness. OBJECTIVE: general condition of the child moderately. Skin and visible mucous membranes are pale. Cardiac impulse is diffused. Boundaries of the relative cardiac dullness extended to the left and right. Heart sounds are muffled, tripartite rhythm "gallop", the focus of two tones of pulmonary artery systolic murmur at apex. Abdomen soft on palpation, the liver 3 cm below the costal arch, the lower edge of thick, moderately painful, spleen not palpable. At 2-day stay in hospital, the boy suddenly appeared heart attack, the feeling of overall weakness, pain in the heart, dizziness. HR-120 per minute, BP -90/55 mm Hg ECG attached.

Answer the following questions: 1. What is the leading clinical syndrome? 2. Provide the differential diagnose according to the main estimated clinical syndrome. 3.Put the previous clinical diagnose. 4.Create the plan of the clinical examination. 5.Create treatment strategy.

B. The tests for self-control

1. Female 15 years old, does not present a complaint. When the planned survey on ECG recorded the correct sinus rhythm with a frequency of 76 min, normal axis position of the heart, interval PQ 0,22 sec, QRS complex is not changed. Your conclusion: A. Complete atrioventricular block B. Blockade of the left foot beam Hisa C. atrioventricular block ΙΙ degree, type Mobitts Ι D. ECG within age norms E. atrioventricular block Ι degree.

2. The examination in 10 year-old boy was found heart rhythm disturbances in the form of beats. Complaints absent. The ECG - sinus rhythm, the vertical position of EAH. The recording part of extrasystoles by type of parasystole (P prong is absent, QRS-complexes deformed T waves negative, full compensatory pause). What kind of beats in a child? A Atrial B. Atrioventricular C. Atrioventricular dissociation D. Ventricular E. Nodal

3. For ECG recording, the boy with severe bradycardia was found that the atria and ventricles in a different rhythm, P wave not associated with complexes QRS, QRS complexes widened (0,12 sec). Revealed changes typical: A. Complete atrioventricular block B. Ventricular C. Atrioventricular block ΙΙ degrees. D. Block of the left bundle branch block E. Glycoside intoxication

4. Female 9 years old, taken to hospital by ambulance complaining of a sudden outbreak of palpitations, general weakness, pain in the heart, a sense of fear. The ECG recorded large (more than 0,1 seconds) QRS complexes with a frequency of 190 per minute, rough, discordant T wave main prongs of the complex QRS, P prong does not recognize. What is the rhythm disturbance occurs in girls? A. Ventricular extrasystole B. Supraventricular paroxysmal tachycardia C. Beats with atrioventricular D. Ventricular paroxysmal tachycardia E. Extrasystoles

5. You have to provide emergency assistance in a fit of supraventricular tachycardia in a child 9 years old. Past vagal test result is not given, specify the dose of ATP, which you enter this child: A. 5 ml B. Of 1 ml C. 0,5 ml D. 2 ml E. 10 ml

6. In the hospital, an ambulance delivered a child of 3 years from attack of ventricular tachycardia. HR 220 per min, a BP 80/40 mm. Hg. . Which of the following drugs is contraindicated for relief of an attack? A. Propranolol B. Aymalin C. Digoxin D. Kordaron E. Izoptin

7. Female 8 years treated in the cardiology department at the Non-rheumatic carditis. At the 3 day hospital stay the child developed an attack of supraventricular tachycardia. How to start emergency care? A. Introduction ATP B. Immediate transport to the intensive care unit C. Digoxin D. Conducting of vagal tests E. Introduction KORDARON

8. Female 17 years complained of transient loss of consciousness, weakness. OBJECTIVE: pale skin, moderate acrocyanosis. Percussion of the heart borders extended to the left. Auscultation - rhythm is incorrect, bradyarrhythmia, auscultated systolic murmur on top. The ECG - sinus rhythm, bradycardia, a gradual increase in the interval P-Q, followed by precipitation of the complex QRS, disturbance of myocardial repolarization. A diagnosis. A. Atrioventricular block ΙΙ degree, type Mobitts ΙΙ B. Complete atrioventricular block C. Atrioventricular block ΙΙ degree. Type Mobitts Ι D. Sinoauricular block E. Atrioventricular block Ι degree

9. In patients with acute rheumatic fever, which occurs with symptoms endomiokardita diagnosed with atrioventricular block ΙΙΙ degree. The next day the patient began to complain of dizziness, feeling of general weakness, and then lost consciousness. Which drug should be used when providing emergency assistance to improve the conduction of AV-node? A. Kordaron B. Adrenaline C. Propranolol D. Atropine E. Digoxin

10. What is clinically manifest violation of the rhythm is common in patients with the syndrome of Wolff-Parkinson-White syndrome. A. Complete atrioventricular block B. Ventricular fibrillation C. Tachicardia D. Atrioventricular block ΙΙ degree, type Mobitts Ι E. Atrioventricular block ΙΙ degree, type Mobitts ΙΙ

11. Female 8 years old, suffers from congenital AV block ΙΙΙ. During the last month frequent syncopal states. At the time of examination in heart rate is 38 beats / min, BP 80/55 mm. Hg, signs of heart failure. The most appropriate tactic for further reference of the patient will: A. Clinical supervision with the re-inspection after 3 months. B. Appointment of kardiometabolitnih C. Appointment of sympathomimetics and follow-up D. Addressing the issue of the pacemaker E. Appointment KORDARON

12. Enter the drug for relief of attacks of ventricular paroxysmal tachycardia: A. Digoxin B. Lidocaine C. Propranolol D. ATP E. Izadrin

13. The boy was a general weakness, dizziness, a history of two syncopal states. On examination the boy pale. Hypertrophied tonsils, loosened. Borders of the heart is not removed. Muted tones, arrhythmic. ECG - sinus rhythm, wrong. HR 82 per minute. PQ - 0,2. Atropine test is negative. Name the cause of syncope in a child: A. Sinus bradycardia B. AV block Ι degree C. Sick sinus syndrome D. Morgagni-Adams-Stokes syndrome E. Vaso-vagal episodes

14. Girl recovering from tonsillitis complains of fever up to subfebrile figures, fatigue, and interruptions in the work of the heart. The left border of the relative cardiac dullness is located at 1 cm to the left of the mid clavicular line. Heart sounds are muffled, arrhythmic. On palpation abdominal pain at the point of Kerala. ECG - voltage reduced. After each ventricular complex recorded expanded and deformed kopmleks QRS with discordantly positioned segment ST. What is the cause of cardiac arrhythmias in this girl? A. Non-rheumatic carditis B. Violation of electrolyte balance C. Viscera - visceral reflexes D. Autonomic dysfunction E. Dyshormonal violations

15. The boy of 13 years, after physical exertion have stabbing pains in his heart, a sense of numbness, as well as the irregularity of the heart. What tests must assign to clarify the cause arrhythmia? A. Electrocardiogram B. Echocardiogram C. Veloergometry D. Cardiointervalography E. X-ray of the chest cavity

16. A child of 9 months, there has for congenital heart disease (ventricular septal defect). Gets digoxin. With a planned inspection of medical attention to arrhythmic heart activity. In electrocardiogram recorded frequent ventricular extrasystoles. What should be the tactics of the physician in this case? " A. Appointment of furosemide B. Cancel digoxin C. Reduced dose of digoxin D. Appointment panangin E. Appointment prpranolol

17. The child suddenly appeared an attack of tachycardia, dyspnoea, stabbing pain in my heart. When ECG - Heart rate 210 per minute. Complex QRS 0,13 seconds., Deformed. Which drug is shown to a child with a view to providing emergency assistance? A. Lidocaine B. ATP C. Korglikon D. Verapamil E. Panangin

18. Boy 17 years, no complaints. On examination of the ECG recorded the correct sinus rhythm with a frequency of 64 a minute, the normal position the axis of the heart, the interval PQ 0,22 sec, QRS complex was not changed. Your diagnosis: A. ECG within the age norm B. Atrioventricular block Ι degree C. Atrioventricular block ΙΙ degree, type Mobitts Ι D. Complete atrioventricular block E. Left bundle branch block

19. Boy 12 years treated at the focal Non-rheumatic carditis. At the 5 day of illness your child is having an attack of supraventricular tachycardia. Enter the drug of choice for emergency care? A. ATP B. Verapamil C. Digoxin D. Propranolol E. Kordaron

20. Boy 7 years old, complained of disruption of the heart, fatigue, and headaches. When an ECG - Heart rate 110 per minute, voltage is not reduced. After each normal QRS complex recorded an expanded and deformed complex QRS. What is the rhythm disturbance occurs in a child? A. Ventricular premature beats, trigeminiya B. Supraventricular extrasystoles C. Paroxysmal ventricular tachycardia D. Ventricular premature beats, bigeminy E. No paroxysmal ventricular tachycardia

21. Boy 12 years complained of irritability, fatigue, dizziness, dimness in his eyes. Yesterday at the gym class first appeared fainting. ECG: voltage is not reduced, the rhythm of the sinus, heart rate 48 per minute, PQ 0,2 sec., Complex QRS 0,1 sec. What dysrhythmia or conduction takes place in a child? A. Sinus bradycardia B. Ι degree atrioventricular block C. ΙΙ degree atrioventricular block D. ΙΙΙ degree atrioventricular block E. Sinus bradyarrhythmia

22.The child is 14 years to eliminate the attack of paroxysmal tachycardia doctor had Valsalva procedure. What is the procedure? A. breath B. Pressure on the root of the tongue C. Delay breathing with straining D. Pressure on the eyeballs E. Carotid sinus massage

23. Child suffering from paroxysmal tachycardia, with a view to coping with an attack appointed verapamil. Which group of drugs on the is this drug? A. Calcium channel blockers B. β - blocker C. Membranostabilizations D. Cardiac glycosides E. Local

24. Boy 13 years complains pritsupy brief loss of consciousness, weakness. The ECG - sinus rhythm, bradycardia, increase interval P-Q with the periodic shedding of the complex QRS, a violation of myocardial repolarization. Diagnosis. A. Atrioventricular block ΙΙ degree, type Mobitts Ι B. Complete AV block C. Atrioventricular block ΙΙ degree, type Mobitts ΙΙ D. Atrioventricular block Ι degree E. Sinoauricular block

Standards replies: 1E, 2D, 3A, 4D, 5В, 6C, 7D, 8C, 9D, 10А, 11D, 12B, 13C, 14A, 15A, 16B, 17A, 18B, 19A, 20D, 21A, 22C, 23A, 24A

7. Literature: Basic 1. Behrman Richard E., Robert M. Kliegman, Hal B. Jenson, eds. Nelson Texbook of Paediatrics. Philadelfia: WB Saunders, 18th edition - 2012. – 994 р. – Р.638. 2. Pediatrics: Textbook for students of higher medical educational institutions III-IV levels of accreditation. 2 edition revised and enlarged. / VG Maidannik. - Kharkov: Folio, 2002. - pp. 237 - 278. 3. Children's Diseases (VM Sidelnykov, V. Berezhnaya, B. J. Resnik, etc.). - K.: "Health", 1999 - pp. 413-423. 4. Selected issues of child cardiorheumatology. Manual for students of higher educational institutions of IV level of accreditation. / Ed. prof. AP Volosovec, VM Sava, SP Nick. - Kyiv, Kharkiv. - 2006. pp. 345-380. 5. Children's Diseases (VM Sidelnykov, V. Berezhnaya, B. J. Resnik, etc.). - K.: "Health", 1999 – pp. 286-306. 6. Belokon NA, Kuberher MB Heart disease and vessels in children. Guid. for doctors - Moscow: "Medicine" - 1987.- ch.2.-pp. 47-136 7. VP Orlov.Guidelines for electrocardiography .- M.MIA.-1997.- 480 pp. Additional 1. Pathological physiology: (textbook-by for medical students. ) / NN Zaikov, Y. Beac, AV Ataman et al. - 3 rd ed.,-K.: "Logos", 1996. - Pp.394-423, 196-223. 2. Propedeutics childhood illnesses and child care: A textbook for students of higher medical educational institutions. - Vinnytsia: GP CCF, 2006 - page 490 - 506. Lectures on the Faculty of Pediatrics 3. Chebotarev VD, VG Maidannyk Propaedeutic Pediatrics. - K., 1999. - Pp. 358-386 Cardiology children and adolescents (favourites issues) / under. Ed. prof. SS Kazak. - Donetsk. - 2004. - Pp. 47-88.

The methodical instructions are compiled by Bubyr L.M.

Methodical instructions were discussed and approved at the meeting of department of Pediatrics №2 protocol № ___ of ______20____.

Additions and changes were made “____” ______20____ Additions and changes were made “____” ______20____ Additions and changes were made “____” ______20____ Additions and changes were made “____” ______20____ Additions and changes were made “____” ______20____ MINISTRY OF HEALTH OF UKRAINE UKRAINIAN MEDICAL STOMATOLOGICAL ACADEMY DEPARTMENT OF PEDIATRICS №2

Approved at the meeting of department of Pediatrics №2 Minutes № 21 " 05 " 06 2020 Head of the department MD, Prof. Kryuchko T.O. ______

METHODICAL INSTRUCTIONS FOR INDEPENDENT WORK OF STUDENTS DURING THE TRAINING FOR THE PRACTICAL (SEMINARS) CLASS

Educational discipline Pediatrics Моdule № 1 Substantial mоdule № 3 Topic of lesson Functional and organic diseases of esophagus and stomach in older children. Course 4 Faculty medical

Poltava 2020

1 1. Actuality of the subject. The diseases of the digestive system are widespread and have significant social value. The structure of general morbidity in our country their frequency is more than 100 children from 1000 and has a tendency to increase. For the past 10 years the prevalence of children gastroenterological pathology increased by 20%. The leading role in diseases of digestive system in children belongs to the affections of the upper part of the intestine tract. High contamination by helicobacter infection, and direct relationship between helicobacter-associated diseases in young age and development of gastric atrophy and cancer will determine the actuality and importance of early detection and adequate treatment of these states.

2. Concrete aims: - Determe the different clinical forms and complications of the digestive system diseases based on complaints, anamnesis and objective test data. - Identify the tactic of the management of patients with digestive diseases. - Demonstrate the ability to manage medical records. - Manage the plan of the examination. - Interpret data of the clinical and laboratory examinations. - Conduct differential diagnosis. - Make a preliminary diagnosis. - Prescribe treatment to the children with ulcer, gastroduodenitis. - Demonstrate knowledge of the moral and deontological principles of medical specialist and professional subordination.

3. Basic knowledge, skills, habits necessary for studying the subject (interdisciplinary integration). Name of previous Acquired skills disciplines Anatomy, physiology Anatomical and physiological features of the digestive system in children. Characterize the performance standards of the digestive system in children Pathological anatomy Know the specifics of the hormonal and neurological regulation of gastric and physiology secretion, digestion, morphological changes in the intestinal mucosa, the mechanism of formation of erosions, the mechanism of development of clinical signs. Interpretation of test results. Propedeutiks childhood Know the methods of investigation of the digestive system in children, diseases instrumental methods of examination of the state of the gastrointestinal tract in children. Spend palpation, percussion, auscultation of the digestive system of the child. Additional surveys, identify and interpret the indicators - FEGDS, pH meters, samples for determination of Hp, ultrasound. Microbiology Know the procedure crops secretions and secrets and interpret the results. Methods for detection H.pylory Pharmacology Knowing the group of drugs used for the treatment of diseases of the digestive tract. Prescribe the necessary drugs, taking into account the state of the child's age and weight, the characteristics of the individual reactions. To be able to write prescriptions. Surgery Know clinicoanamnestic complex signs of complications of diseases of the digestive system. Fundamentals of surgical treatment. Faculty Pediatrics Clinical-anamnestic and laboratory-instrumental complex features gastrointestinal diseases in children of different ages. Identify signs of organic and functional diseases of the gastrointestinal tract in children of different ages. Intradisciplinary Give a clinical evaluation of changes in clinical, biochemical and integration immunological studies of blood serum, to interpret the data of instrumental methods of examination.

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4. The tasks for independent work during preparation for the study:

4.1. The list of basic terms, parameters, characteristics, which the student should master while training for the studies: Term Definition Heartburn So when acid backs up into your esophagus, it causes an uncomfortable burning sensation that rises from the stomach or lower chest up towards the neck. In some cases, stomach acid may be regurgitated into your mouth, causing an unpleasant sour taste. Gastroesophageal Most people experience heartburn occasionally, but some suffer from it more reflux disease regularly. If you suffer from heartburn or other reflux symptoms that you find troublesome, you may have reflux disease or gastroesophageal reflux disease. Chronic Chronic recurrent inflammatory disease which accompanied by structural gastroduodenitis change of the mucus apparatus and secretory and motor disorders.

Ulcer Polyetiological disease which characterized by the formation of ulcer in the stomach and duodenum. Monihame’s syndrom Reducing of the pain after eating and the appearance of the pain in1,5-2 hours after eating. Melena Black colour of the fecus (result of the bleeding from upper gastrointestinal tract). Vomiting Discharge of stomach contents from the upper intestine through the mouth. Nausea Discomfort in the epigastric area. Pyrosis Burning sensation behind breast bone. Eructation Sudden gas or gastric contents revenues from the stomach. Mendel’s syndrom Pain in the Shoffar’s area after percussion (positive at duodenitis, duodenum ulcer). Fibrogastroscopy The method of the research of the esophagus, stomach and duodenum with fibroscope. рН- measuring Defining of the acidproduction function of the stomach. Coprograme Results of macroscopic and microscopic study of the fecus. Gregersen test Analysis of the fecus to the latent blood. Helicobacter pylori The main etiologic factor of the gastritis and ulcer disease.

4.2. The theoretical questions to the studies: 1. What is heartburn? 2. What causes reflux? 3. What is reflux disease or GERD? 4. What are the symptoms of reflux disease? 5. How can GERD affect your life? 6. What is esophagitis? 7. Can GERD lead to more serious problems? 8. How is GERD diagnosed? What is an endoscopy? 9. How is GERD treated? What if you are not happy with the treatment you have been prescribed? 10. What can you do to help to reduce your GERD symptoms? 11. Is surgery the answer for GERD? Can dysphagia be helped by surgery? 12. What is a peptic ulcer? 13. How do you get a peptic ulcer? 14. What are the symptoms of a peptic ulcer? 15. How is a peptic ulcer diagnosed? 16. What is an endoscopy?

3 17. How is the presence of H. pylori detected? 18. How serious are peptic ulcers? 19. What is the treatment for peptic ulcers that are associated with H... 20. What about surgery for a peptic ulcer? 21. Are some peptic ulcers not associated with H. pylori infection?

4.3. The practical works (tasks) which are done at the studies: – To collect complaints, anamnesis of the disease. – To conduct an objective examination of the patient. – To assess results of laboratory and instrumental methods. – To make on a preliminary diagnosis. – To perform a differential diagnosis. – To assign treatment according to the protocol. – To make out a plan of rehabilitation. – To write out recipes.

5. The contents of the topic:

Gastroesophageal reflux disease (GERD) When you eat or drink something, the food passes from your mouth into your esophagus, then into your stomach (Figure 1), and on into the rest of the digestive system.

Figure 1. The esophagus Figure 2. The lower esophageal sphincter acts as a one-way valve to let food into the stomach but not to exit the same way

There is a valve mechanism at the top of your stomach called the lower esophageal sphincter or LES (Figure 2), which acts as a one-way valve, opening to let the food into the stomach and then closing to prevent the contents of the stomach from moving back into the esophagus.

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Sometimes this valve is not completely effective and allows acidic stomach juices to flow back into the esophagus (Figure 3) – a condition known as reflux or gastroesophageal reflux. Exposure to harsh stomach acid irritates the sensitive lining of the esophagus, causing the unpleasant sensation referred to as heartburn. In some cases, stomach acid may be regurgitated into the mouth, causing an unpleasant sour taste. Other symptoms may include pain and difficulty in swallowing, belching or , persistent coughing or hoarseness, upper abdominal pain (epigastric pain) or chest pain.

Figure 3. If the LES does not work correctly, acid is allowed to pass back into the esophagus If you suffer from reflux, you may find that certain things make it worse, including: • Food o Certain foods or drinks, such as fatty or highly spiced food, onions, alcohol, fruit juices, coffee and chocolate. o Large meals. o Eating too close to bedtime – a stomach full of food and acid is more prone to reflux, especially when you are lying down. • Smoking • Pregnancy, which increases pressure within the abdomen, pushing the stomach contents into the esophagus. • Medical conditions o If the valve mechanism in the LES does not work properly. o If the stomach produces too much acid, it will be more likely that acid may leak into the esophagus. o Delayed stomach emptying – some people have stomachs that empty their contents into the intestines very slowly. o Patients with hiatal (where a small part of the stomach protrudes beyond the diaphragm into the chest) have an increased risk of suffering from reflux. o People who are obese are more likely to suffer from reflux. • Some medicines can affect the LES so that it does not work properly. These include theophylline, oral contraceptives, benzodiazapines and calcium channel blockers. • Wearing tight clothes around the stomach • Exercise or bending Most people experience heartburn occasionally, but some suffer from it more regularly. If you suffer from heartburn or other reflux symptoms that you find troublesome, you may have reflux disease or gastroesophageal reflux disease (GERD) and you should consider consulting your doctor. Heartburn is the most common symptom of reflux disease (also called gastroesophageal reflux disease or GERD). Heartburn is usually felt as an uncomfortable burning sensation that rises from the stomach or lower chest up towards the neck. Other symptoms include: • pain and difficulty in swallowing • belching or burping • persistent coughing, particularly at night • persistent hoarseness, sore throat and throat clearing • upper abdominal pain (upper stomach pain), also called epigastric pain • chest pain that is not related to heart problems 5 GERD may also play a role in the development of chronic sinus problems, asthma and wheezing. If you suffer regularly from reflux symptoms more than twice a week and find that these interfere with your life, you should think about consulting your doctor. The good news is that medication is available to provide freedom from these symptoms. Over time, refluxed acid may damage the lining of the esophagus, leading to a condition called esophagitis. Esophagitis is an inflammation of the lining of the lower end of the esophagus. • In most people, esophagitis is caused by the digestive juices in the stomach repeatedly moving upwards into the lower esophagus. • In severe cases, the lining of the esophagus may become ulcerated, leading to pain and possible narrowing due to scarring. This may lead to difficulty in swallowing (a condition called dysphagia), first of solid foods and then even of more liquid foods. • Doctors know that approximately half of their patients who have gastroesophageal reflux disease (or GERD) also have esophagitis. For most people with gastroesophageal reflux disease (or GERD), the main problem is the symptoms that may result in a poor quality of life and decreased productivity with regard to work and social activities. • Acid reflux can, over time, cause the inflammation and damage to the esophagus that is called esophagitis. • In severe cases, the lining of the esophagus may become ulcerated, leading to pain and possible narrowing due to scarring. This may result in difficulty in swallowing (a condition called dysphagia), first of solid foods and then even more liquid foods. • Esophagitis may also result in bleeding from the esophagus. If you experience red or black faeces (blood in your stools when you go to the toilet), cough up blood, or suffer from anaemia, you should speak to your doctor. • Severe, long-standing GERD can cause a condition known as Barrett’s esophagus, where the normal lining of the esophagus is replaced by a lining more like that of the stomach or intestine. It is thought that this replacement may be an attempt by the body to protect itself from further injury by acid. • Untreated GERD may also increase the risk of more serious complications, such as esophageal cancer, in a small number of people. In a few cases, your doctor may arrange for further investigations, such as an endoscopy, , to discover whether the lining of the esophagus is affected and you have reflux esophagitis. However, the medications used in the treatment of esophagitis are the same as those recommended when esophagitis is not present. During an endoscopy, a narrow, flexible tube with a fibre-optic light and camera at the end, is placed into the mouth, then the throat and esophagus (the upper part of the digestive system). This allows the doctor to see the inside of the esophagus to look for any reddening or sores on the esophageal wall. It is also possible to take a small sample (a biopsy) to check for any abnormalities.

Fortunately, heartburn and gastroesophageal reflux disease (GERD) can be treated by making the stomach contents less acid. This reduces the discomfort caused by refluxed acid and enables the lining of the esophagus to heal. Drug treatment is often only needed for a short time period, although it usually needs to be represcribed. 1. Proton pump inhibitors (PPIs). Proton pump inhibitors are a group of medicines that includes esomeprazole, lansoprazole, omeprazole, pantoprazole and rabeprazole. • Proton pump inhibitors provide rapid relief from heartburn and the other symptoms of GERD and are highly effective in healing esophagitis. • These medicines block the action of the stomach’s proton pumps, which are located in parietal cells in the stomach walls (Figure 1). The proton pumps pump hydrogen ions into the stomach, making the stomach acidic.

6 • By blocking the proton pump, proton pump inhibitors reduce the acidity in the stomach. They therefore reduce the acidity of the stomach contents leaking back into the esophagus, which reduces the incidence of heartburn and other acid reflux symptoms. • Proton pump inhibitors are available from your doctor on prescription and, in some countries, can also be bought over the counter from a pharmacist. Figure 2. Proton pump inhibitors block the final step of acid production in the stomach 2. H2 antagonists Histamine2 (H2)-receptor antagonists are a group of drugs that includes cimetidine, nizatidine and ranitidine. • These medicines work by stopping (blocking) the action of histamine (a chemical released by the stomach). The presence of histamine in the stomach results in the release of hydrogen ions, which makes the stomach more acidic. • H2-receptor antagonists only partially block acid production in the stomach and are therefore less effective than PPIs in reflux disease. • Many H2 antagonists can be bought over the counter at low doses, but a doctor’s prescription is needed for higher doses. Other medicines (called pro-kinetic agents) increase the movement of the stomach. They work by increasing the pressure of the lower esophageal sphincter (the point where the esophagus joins the stomach) and promote emptying of the stomach. 3. and alginates There are a number of over-the-counter preparations, such as antacids or antacid-alginates, which can be taken to relieve occasional heartburn. These remedies, however, do not reduce acidity in the stomach sufficiently to help the lining of the esophagus to heal and are not usually recommended to treat the frequent heartburn suffered by people with GERD. Antacids provide relief from heartburn by neutralising the acid in the stomach. Their main ingredients are: • • Magnesium salts • Calcium carbonate • Sodium bicarbonate • Potassium bicarbonate • Bismuth salts Antacids sold over the counter may contain one or a combination of these products. For example, aluminium and magnesium salts are often combined to reduce the incidence of diarrhoea or constipation. Antacids are best taken when the symptoms of heartburn first occur, preferably about 1 hour after a meal. However, antacids may interfere with the absorption of other medicines from the digestive system into the blood, so a gap of at least 2 hours should be left between using an antacid and taking other medicines. Some antacids contain sodium alginate. Sodium alginate forms a ‘raft’ that floats to the top of the stomach, forming a barrier between the acid and the esophagus, thus preventing acid reflux into the esophagus. Your symptoms may be provoked by particular foods: • Avoid eating too close to bedtime (try to leave at least 2–3 hours before you go to bed) • Avoid lying down after meals • Eat small, regular meals • Avoid tight clothing that places pressure on your stomach • Lose weight, if necessary • Give up smoking • Raising the head of the bed by 20 cm (4–6 inches) may help to reduce night-time reflux.

Surgery, using a procedure known as ’fundoplication’, is an appropriate choice for a small number of patients with gastroesophageal reflux disease (GERD). Fundoplication was originally done as open surgery, which involved making an incision into the abdomen under general anaesthesia. However, fundoplication has become much more popular since the development of laparoscopic techniques (also known as minimally invasive or keyhole surgery), which offer faster recovery and fewer complications. While the vast majority of patients will be

7 deemed suitable for laparoscopic surgery, a few will not, such as those who have had previous abdominal surgery, or individuals with certain other medical conditions. How fundoplication works In fundoplication, the surgeon creates a new ’valve’ between the esophagus and the stomach by wrapping the upper portion of the stomach around the lowest portion of the esophagus. As the stomach becomes distended after eating, the wrap compresses the esophagus, preventing stomach acid from escaping. If the patient has other problems – for instance, a hiatal hernia, a swallowing disorder, or a shortened esophagus – these may also be corrected during surgery. Fundoplication is a relatively safe operation The most common complication of fundoplication is perforation of the stomach or esophagus, which occurs in around 1 in 100 patients. Rarely, perforation or bleeding will be missed during the procedure and the patient will have to undergo a second operation. In addition, around 5% of patients will start off being treated laparoscopically but will then be converted to open surgery. Surgery versus drugs Fundoplication has become an increasingly popular treatment for GERD. Exponents of surgery believe that it removes the need for drug therapy and reduces the risk of esophageal cancer. Indeed, a study conducted in the late 1980s initially appeared to confirm the superiority of surgery over drug therapy for controlling the signs and symptoms of GERD. However, 10 years on, the researchers revisited the study participants and were surprised to discover that almost two-thirds of the surgery patients still needed medication to control their symptoms. In addition, patients who had undergone surgery a decade previously were just as likely to have developed esophageal cancer as those who only received medication. A controversial issue On the basis of their findings, and from weighing up the relative risks, benefits, and financial costs of surgery and drug therapy, the researchers concluded: “Long-term medical therapy with proton pump inhibitors is the preferred strategy for patients with GERD and severe esophagitis.” Most experts agree that there are some patients in whom surgery may be preferable to drugs – for instance, individuals who are unable to tolerate proton pump inhibitors due to side effects, patients who develop additional symptoms such as cough, chest pain, or hoarseness, or those who completely respond to drug therapy but relapse with symptoms when medication is withdrawn. But, with the advent of more effective and better-tolerated drugs, the advantages of surgery appear to be less clear-cut for the majority of patients with GERD.

• Guided wire – Dilators are passed over a thin wire, which is positioned in the narrowed tube. • Balloons – Long, narrow balloons can be passed into the esophagus and then gently inflated to open up the narrowed tube. • Bougie – A series of increasingly larger, soft rubber or plastic dilators are placed over the narrow part of the esophagus, gently widening the tube.

Patients with swallowing difficulties will normally obtain complete relief after one of these procedures. During, or after the procedure you may experience a small amount of bleeding from the esophagus. Complications from the procedure, such as excessive bleeding due to damage to the esophagus, are rare.

Peptic ulcer disease (PUD) The lesion of peptic ulcer disease (PUD) is a disruption in the mucosal layer of the stomach or duodenum. An ulcer is distinguished from an erosion by its penetration through the muscularis mucosa or the muscular coating of the gastric or duodenal wall. PUD results from the imbalance between defensive factors that protect the mucosa and offensive factors that disrupt this important barrier. Some mucosal protective factors include the water-insoluble mucous gel layer, local production of bicarbonate, regulation of gastric acid secretion, and adequate mucosal blood flow. Aggressive factors include the acid-pepsin environment, infection with Helicobacter pylori, and mucosal ischemia.

8 Primary peptic ulcers are still relatively uncommon in children and account for roughly 1 in 2500 pediatric hospital admissions. Primary ulcers are seen more often in adolescents than in children and tend to recur after initial healing. Although affected children are thought to have high acid secretion, this has not been proven. Many of the primary ulcers seen in teenagers are now thought to be associated with H pylori infection. Secondary ulcers are seen in head trauma, severe burns, and in use of corticosteroids and nonsteroidal anti-inflammatory drugs (NSAIDs).

Pathophysiology. The 2 most important concepts in understanding the pathophysiology of PUD in children are the host factors that serve to protect the GI mucosa from ulceration and the inflammatory mediators and aggressive factors that contribute to mucosal inflammation and ulceration. An overlying physiochemical barrier provides cytoprotection of the gastric mucosa. This barrier comprises water-insoluble gastric mucus, gastrically produced bicarbonate, an unstirred water layer, phospholipids, rapid shedding of cells resulting from epidermal growth factor, normal mucosal blood flow, prostaglandin-stimulated bicarbonate, mucus production, and inhibited acid secretion. Contributors to mucosal inflammation and ulceration include endogenous factors, such as gastric acidity, acid-dependent pepsin, and mucosal ischemia, as well as exogenous factors, such as drugs (eg, NSAIDs, aspirin, corticosteroids), alcohol, cigarette smoking, corrosive chemicals (eg, lye), and emotional stress. In patients with traumatic injuries, burns, sepsis, respiratory failure, or other critical systemic illnesses, many factors can contribute to erosions and ulcers, including mucosal ischemia, increased gastric acid and pepsin production, higher levels of endogenous catecholamines and steroids, and decreased prostaglandins and mucus production. Important mediators of mucosal inflammation and resultant ulceration include oxygen free radicals, lymphokines, and monokines. The gram-negative spirochete, H pylori, was first linked to gastritis in 1983. Since then, further study of H pylori has revealed that it is a major part of the triad, which includes acid and pepsin, that contributes to primary PUD. The unique microbiologic characteristics of this organism, such as urease production, allows it alkalinize its microenvironment and survive for years in the hostile acidic environment of the stomach, where it causes mucosal inflammation and, in some individuals, worsens the severity of PUD. When H pylori colonizes the gastric mucosa, inflammation usually results. The casual association between H pylori gastritis and duodenal ulceration is now well established in the adult and pediatric literature. In patients infected with H pylori, high levels of gastrin and pepsinogen and reduced levels of somatostatin have been measured. In infected patients, exposure of the duodenum to acid is increased. Virulence factors produced by H pylori, including urease, catalase, vacuolating cytotoxin, and lipopolysaccharide, are well described. However, the specific roles of these factors and the secretory disturbances associated with H pylori infection in subsequent duodenal ulceration remains unclear. • Primary PUD is uncommon in infants and in children younger than 10 years. The prevalence of primary PUD increases during adolescence. • Secondary PUD can affect patients of all ages, but its prevalence is increased in patients younger than 6 years. History. • In children in whom peptic ulcer disease (PUD) is suspected, include the following in the history: o Review of past illnesses and chronic medical conditions o Family history of ulcer disease, including known H pylori infection, or conditions affecting the GI tract (eg, Crohn disease) o Character, location, frequency, duration, severity, and exacerbating (especially meals in children) and alleviating factors of abdominal pain o Vomiting and description of gastric material o Bowel habits and description of stool (eg, profuse diarrhea seen in Zollinger-Ellison syndrome [ZES]) o Prescribed and over-the-counter (OTC) medications, especially NSAIDs and corticosteroids o Prior diagnostic testing and specific GI therapies o Appetite, diet, and weight changes 9 o Family and social stressors o Alcohol ingestion and smoking habits • Abdominal pain is the most common symptom of childhood PUD. o The pain is usually dull and vague. The pain is most likely to be dull and aching rather than sharp and burning, as adults describe. Food intake often causes the pain to worsen; this is the opposite of the adult pattern. o The pain may be poorly localized or localized to the periumbilical or epigastric areas. o In preschool-aged children, the pain is typically periumbilical and worsens after eating. o After the age of 6 years, the child's description of pain may be similar to the description by adults. The classic pain of PUD (ie, pain that awakens the child, worsens with food, and is relieved by fasting) is described infrequently, but it helps in distinguishing GI pathology from psychogenic pathology when present. o Frequent exacerbations and remissions of pain extend over weeks to months. • Vomiting in infants and toddlers may be associated with slow growth. Recurrent vomiting is also noted in preschool- and school-aged children. • GI tract bleeding (eg, melena, hematochezia, hematemesis) may be another presentation in children. o In infants and particularly neonates, serious underlying illness and stress ulceration most commonly manifest as acute perforation or hemorrhage. o GI bleeding may lead to iron-deficiency anemia (IDA), and patients may present with vague complaints of fatigue, headache, dyspnea, or malaise. • For children with ulcer perforation, the symptoms are consistent with peritonitis and abrupt in onset. Physical. • Include the following in the physical examination: o Observation of the general appearance of the child o Evaluation of vital signs o Assessment of perfusion with attention to mental status, heart rate, pulses, and capillary refill o Assessment of hydration status with attention to moisture of the mucous membranes and skin turgor o Observation of any pallor of the skin and conjunctivae o Thorough chest examination o Careful inspection, auscultation, and palpation of the abdomen, with notation of any liver or spleen enlargement o Rectal examination and stool guaiac testing o Pelvic examination in sexually active female patients with pain o Examination of the testicles and inguinal area in male patients • Hemorrhage accompanies PUD in 15-20% of patients. • resulting from perforation of the GI tract occurs in 5% of children with PUD. Causes. • Primary PUD o Genetic factors may be important, as indicated by the observation that as many as 50% of children with PUD have a first- or second-degree relative with PUD. In addition, a concordance rate that is 3 times higher in monozygotic than in dizygotic twins has been described, and children with blood group O have an increased incidence of PUD. o Emotional stress has been described as a factor predisposing children to PUD. o Alcohol has been documented to produce inflammation, erosions, and hemorrhage in the gastric mucosa in animal and adult human studies. Caffeine intake also predisposes children to PUD. o Clinical and laboratory data provide strong evidence that H pylori infection causes chronic gastritis and primary duodenal ulcer disease. H pylori gastritis has not been strongly associated with gastric ulceration in children.

10 o Compared with people who do not smoke cigarettes, those who do are twice as likely to develop PUD. Smoking may lead to ulceration, slow healing, and an increased risk of recurrent disease. • Secondary PUD o Corticosteroids, NSAIDs, and aspirin use predispose children to stress ulceration. These drugs disrupt the mucosal permeability barrier, rendering the mucosa vulnerable to injury. As many as 30% of adults taking NSAIDS have GI adverse effects. Although the prevalence of NSAID gastropathy in children is unknown, it seems to be increasing, especially in children with chronic arthritis treated with NSAIDS. Recent case reports have demonstrated gastric ulceration from low-dose ibuprofen in children, even after 1 or 2 doses. o Serious systemic illness, sepsis, hypotension, respiratory failure, and multiple traumatic injuries increase the risk for secondary (stress) ulceration. . The ulcer associated with a brain tumor or injury, or Curling ulcer, is characterized as single, deep, and prone to perforation. It is associated with high gastric acid output, and it is located in the duodenum or stomach. . Extensive burns are also associated with ulcers, namely Curling ulcers. . Stress ulceration and upper-I hemorrhage are complications being encountered more often than before in critically ill children in the intensive care setting. Severe illness and a decreased gastric pH, are related to an increased risk of gastric ulceration and hemorrhage. Neutralization of gastric acid inactivates proteolytic pepsin, which is responsible for gastric mucosal injury. Therefore, gastric pH of critically ill children should be maintained at more than 6 to prevent injury. ZES is a rare disorder that can cause gastric or duodenal ulcers, usually multiple, from excessive acid secretion. ZES should be suspected if the patient has severe peptic ulceration, kidney stones, watery diarrhea or malabsorption. ZES can also be associated with multiple endocrine neoplasias type I, which occurs at an age earlier than does isolated ZES. Patients with ZES usually have fasting serum gastrin levels of more than 200 pg/ml and basal gastric acid hypersecretion at more than 15 mEq/h. Protein pump inhibitor (PPI) therapy should be discontinued at least 2 weeks before the gastrin level is measured. Because treatment protocols may be different for different types of ulcers, it is important to adequately diagnose ulcer disease and H. pylori before starting treatment. For example, for an NSAID- induced ulcer, treatment is quite different from the treatment for a person diagnosed with an ulcer caused by the bacterium, H. pylori. In addition to a complete medical history and physical examination, diagnostic procedures for ulcers may include:

• upper GI (gastrointestinal) series - examination of the esophagus, stomach, and duodenum (the first section of the ) with an endoscope (a small, flexible tube with a light and a camera lens at the end). • endoscopy - a test that uses a small, flexible tube with a light and a camera lens at the end (endoscope) to examine the inside of part of the digestive tract. Tissue samples from inside the digestive tract may also be taken for examination.

11

• blood, stool, breath, and stomach tissue tests - performed to detect the presence of H. pylori. Although some of the tests for H. pylori may occasionally give false-positive results, or may give false-negative results in people who have recently taken antibiotics,omeprazole, or bismuth, research shows these tests can be accurate in detecting the bacteria.

Specific treatment will be determined by your child's physician based on the following: • your child's age, overall health, and medical history • the extent of the disease • your child's tolerance for specific medications, procedures, or therapies • the expectations for the course of the disease • your opinion or preference Recommended treatment may include:  Lifestyle changes In the past, physicians advised people with ulcers to avoid spicy, fatty, or acidic foods. However, a bland diet is now known to be ineffective for treating or avoiding ulcers. No particular diet is helpful for most ulcer patients. If it seems that certain foods cause irritation, please discuss the problem with your child's physician. Some children and teenagers smoke, with or without their parent's knowledge or permission. Smoking has been shown to delay ulcer healing and has been linked to ulcer recurrence.  Medications Physicians may treat stomach and duodenal ulcers with several types of medications, including the following: • H2-blockers -- to reduce the amount of acid the stomach produces by blocking histamine, a powerful of acid secretion. • Proton pump inhibitors -- to more completely block stomach acid production by stopping the stomach's acid pump -- the final step of acid secretion. • Mucosal protective agents -- to shield the stomach's mucous lining from the damage of acid, but do not inhibit the release of acid. When treating H. pylori, these medications or procedures are often used in combination with antibiotics.  Surgery In most cases, anti-ulcer medicines heal ulcers quickly and effectively, and eradication of H. pylori prevents most ulcers from recurring. However, people who do not respond to medication or who develop complications may require surgery. At present, standard open surgery is performed to treat ulcers. Types include: • Vagotomy -- A procedure that involves cutting parts of the vagus nerve (a nerve that transmits messages from the brain to the stomach) to interrupt messages sent through it, therefore, reducing acid secretion. • Antrectomy -- An operation to remove the lower part of the stomach (antrum), which produces a hormone that stimulates the stomach to secrete digestive juices. Sometimes a surgeon may also remove an adjacent part of the stomach that secretes pepsin and acid. A vagotomy is usually done in conjunction with an antrectomy. • Pyloroplasty - A surgical procedure that may be performed along with a vagotomy, in which the opening into the duodenum and small intestine (pylorus) are enlarged, enabling contents to pass more freely from the stomach.

6. The materials for self-control A. Sample case report The 14 years old boy suffers from chronic gastroduodenitis from 9 years. Last year he marks that deterioration became irritable, sleep is restless, clear seasonality of exacerbations appeared, there

12 is moynihan rhythm pain. The last 2 weeks intense paroxysmal epigastric pain and pyloroduodenal area with irradiation in the back overcome, can be night pain, fasting pain, late pain, sometimes vomiting occurs without admixture of blood. Objectives: 1. Set the clinical diagnosis. 2. Determine the treatment plan. B. The tests for self-control Question: 1 A 2-year-old boy is brought to your office for evaluation of bloody diarrhea, abdominal pain, and vomiting. His mother states that he had a cold 3 days before the symptoms began and that the pain occurs every 20 to 30 minutes. Between episodes of pain, the child falls deeply asleep and is difficult to arouse. Of the following, the MOST appropriate test for the management of this child is A. abdominal ultrasonography B. air contrast C. D. computed tomography of the abdomen E. laparoscopy

Question: 2 A 7-year-old girl who has juvenile rheumatoid arthritis has had a good response to ibuprofen 40 mg/kg per day since being diagnosed 4 months ago. During the past 2 weeks, she has complained of a daily stomachache after school and has not been interested in her usual snack. Physical examination reveals no abdominal tenderness to palpation. Stool examination for occult blood is negative. Of the following, the MOST appropriate management at this time is to A. continue ibuprofen and add omeprazole B. continue ibuprofen and add ranitidine C. continue ibuprofen and add sucralfate D. discontinue ibuprofen and begin naproxen E. discontinue ibuprofen and begin tolmetin

Question: 3 A 10-year-old girl is receiving nonsteroidal anti-inflammatory therapy for juvenile rheumatoid arthritis. She comes to your office complaining of severe epigastric abdominal pain and vomiting. Of the following, the MOST likely cause of her symptoms is A. gastroesophageal reflux disease B. Helicobacter pylori-associated gastritis C. irritable bowel syndrome D. E. peptic ulcer disease

Question: 4 A 4-week-old boy presents with a 2-week history of vomiting his formula. His mother describes the vomiting as forceful, occurring shortly after each feeding, and containing only formula. Bowel movements have been infrequent. Physical examination reveals a dehydrated infant who is eager to feed. Of the following, the MOST likely diagnosis is A. allergic reaction to formula B. gastroenteritis 13 C. gastroesophageal reflux D. pyloric stenosis E. small bowel obstruction

Question: 5 You are evaluating an 8-year-old boy who has had intermittent periumbilical pain of 3 months' duration. Physical examination findings are normal. Helicobacter pylori (H pylori) antibody is elevated. Of the following, a TRUE statement is that A. During treatment for H pylori infection, patients who have gastroesophageal reflux may experience symptomatic improvement. B. Patients who have positive serology for H pylori should be treated with omeprazole and a single antibiotic. C. The patient probably has a duodenal ulcer. D. The prevalence of H pylori infection is increased in areas of high socioeconomic status. E. The specificity of the H pylori serology in children is less than 70%.

Question: 6 A 15-year-old girl presents with her third episode of severe epigastric pain in the past 8 months. In between episodes, she is completely healthy. In each of her prior episodes, amylase and lipase concentrations both were elevated (>500 U/L), but returned to normal within 96 hours. The girl states that she does not consume alcohol and takes no medications. A brother has been hospitalized with similar symptoms. On physical examination, the girl appears well nourished but uncomfortable and is experiencing moderate epigastric tenderness. Complete blood count, transaminases, and bilirubin results are normal; the amylase level is 700 U/L and lipase level is 1,100 U/L. You admit the girl to the hospital for hydration and pain control. Abdominal ultrasonography shows no gallstones, and the common bile duct is not dilated. The pancreas is slightly enlarged, but there is no pseudocyst. Of the following, the MOST appropriate next test to identify the cause of this girl’s illness is A. esophagogastroduodenoscopy B. genetic testing for Johanson-Blizzard syndrome C. mumps antibody titer D. sphincter of Oddi manometry E. sweat test for cystic fibrosis

7. REFERENCES Basic Literature 1. Behrman Richard E., Robert M. Kliegman, Hal B. Jenson, eds. Nelson Texbook of Paediatrics. Philadelfia: WB Saunders, 18th edition - 2012. – 994 р. – Р.638. 2. Buryak V.N., Makhmutov R.F., Poshehonova J.V. Children`s diseases. – Donetsk: Nord-Press, 2010. – 328 p. 3. Pediatry. Guidance aid / Ed. by O. Tiazhka – Kyiv: Medicine, 2007. 4. Children's Diseases (V.M Sidelnykov, V. Berezhnaya, B.J. Resnik, etc.). - K.: "Health", 1999. 5. Medical childhood / gen.ed.prof. Moschych S.K.: Health, 1994.

6. Уніфіковані клінічні протоколи медичної допомоги дітям із захворюваннями органів травлення.- Наказ Міністерства охорони здоров'я України .№ 59 від 29.01.2013 р. 7. Болезни органов пищеварения у детей Майданник В.Г.К.: СП Інтертехнодрук», 2010.- 1157 c. 8. Бєлоусов Ю.В. Гастроентерологія дитячого віку.-Київ, 2007.-438с. 9. Майданник В.Г., Корнейчук В.В., Хайтович Н.В., Салтыкова Г.В. Заболевания пищевода, желудка и двенадцатиперстной кишки у детей.К.: ВБ «Аванпост-Прим», 2008.- 432 c. 14 10. Майданник В.Г., Корнейчук В.В., Хайтович Н.В., Салтыкова Г.В. Заболевания гепатобилиарной системы и поджелудочной железы у детей. К.: ВБ «Аванпост-Прим», 2009.- 409 c. 11. Майданник В.Г., Корнейчук В.В., Хайтович Н.В., Салтыкова Г.В. Заболевания кишечника у детей. : ВБ «Аванпост-Прим», 2009.- 487 c. 12. Ситуаційні завдання з педіатрії/ За ред. чл.-кор. АМН України, проф. В.Г. Майданника. - К.: ВБ „Аванпост-Прим”, 2006. - 206 с. 13. Педиатрия. (ч. 1). Т.А.Крючко, Н.Н.Пеший. Учебное пособие. Полтава, 2010 г. 261 с. 14. Педиатрия. (ч. 2). Т.А.Крючко, Н.Н.Пеший. Учебное пособие. Полтава, 2010 г. 261 с. 15. Педиатрия. Крючко Т.А., Пеший Н.Н. Клинические лекции. ООО НПП «Укрпромторгсервис», Полтава, 2011. 47 с. 16. Тестові завдання з педіатрії /За ред. чл.-кор. АМН України, проф. В.Г. Майданника. - К.: СП „Інтертехнодрук”, 2007. - 429 с. 17. Ю.В.Бєлоусов, Л.Г.Волошина, Н.В.Павленко та ін. Захворювання органів травлення у дітей (стандарти діагностики та лікування). - Харків: ВД „ІНЖЕК”, 2007.- 123с.

Additional: 1. Illustrated Textbook of Paediatrics/ Ed.: T. Lissauer, G. Clayden.- Edinburg: Mosby, 2006.- 410 pp. 2. Forfar & Arneil’s Textbook of Pediatrics/ Ed.: N.Mc Intosh, P.J. Helms, R.L. Smyth, S. Logan.- Edinburg: Churchill Livingstone, 2008. - 1687 pp. 3. Hart JJ. Pediatric gastroesophageal reflux. Am Fam Physician 2006;54:2463-72. 4. Mezoff AG, Balistreri WF. Peptic ulcer disease in children. Pediatr Rev 2005;16:257-65. 5. Kliegman: Nelson Textbook of Pediatrics, 18th ed. Copyright © 2007 Saunders, An Imprint of Elsevier /Chapter 320 – Gastroesophageal Reflux Disease (GERD)/ Susan Orenstein John Peters Seema Khan Nader Youssef Sunny Zaheed Hussain. 6. Kliegman: Nelson Textbook of Pediatrics, 18th ed. Copyright © 2007 Saunders, An Imprint of Elsevier / Chapter 332 – Peptic Ulcer Disease in Children/ Samra S. Blanchard Steven J. Czinn. 7. Hassall E: Clinical practice guidelines for suspected peptic ulcer disease in children. BC Med J, 2001; 36(8): 538-539. 8. Hart JJ. Pediatric gastroesophageal reflux. Am Fam Physician 2006;54:2463-72. 9. Mezoff AG, Balistreri WF. Peptic ulcer disease in children. Pediatr Rev 2005;16:257-65. 10. Крючко Т.А., Пєший Н.Н. Врачебный практикум учебное пособие Полтава ТОВ НВП Укрпромторгсервис 2013 – 177 с. 11. Крючко Т.А., Пєший Н.Н. Практикум семейного врача учебное пособие Полтава ТОВ НВП Укрпромторгсервис 2014 – 274 с. 12. Крючко Т.А., Пєший Н.Н. Практикум для педиатра и семейного врача учебное пособие Полтава ТОВ НВП Укрпромторгсервис 2015 – 249 с. 13. Крючко Т.А., Пєший Н.Н. Практическая педиатрия учебное пособие Полтава ТОВ НВП Укрпромторгсервис 2014 – 232 с. 14. Педіатрія : національний підручник : у 2 т. / Д. Д. Іванов, С. В. Кушніренко, Д. А. Сеймівський [та ін.] ; за ред. В. В. Бережного ; Асоціація педіатрів України. – К. : Сторожук О. В., 2013. – Т. 2. – 1021 с.

The methodical instructions are compiled by Bubyr L.N.

Methodical instructions were discussed and approved at the meeting of department of Pediatrics №2 protocol № ___ of ______20____.

Additions and changes were made “____” ______20____ Additions and changes were made “____” ______20____

15 Additions and changes were made “____” ______20____ Additions and changes were made “____” ______20____

16 MINISTRY OF HEALTH OF UKRAINE UKRAINIAN MEDICAL STOMATOLOGICAL ACADEMY DEPARTMENT OF PEDIATRICS №2

Approved at the meeting of department of Pediatrics №2 Minutes № 21 " 05 " 06 2020 Head of the department MD, Prof. Kryuchko T.O. ______

METHODICAL INSTRUCTIONS FOR INDEPENDENT WORK OF STUDENTS DURING THE TRAINING FOR THE PRACTICAL (SEMINARS) CLASS Academic discipline Pediatrics Моdule № 1 Content mоdule № 3 Functional and organic diseases of hepatobiliary system The topic of the studies in children. Course (year) 4 Faculty Medical

Poltava 2020

1. Actuality of topic: Researches of the last years, based on the most modern and more reliable methods of inspection of gastrointestinal tract, marked to wide distribution of diseases of bile-excreting ways at children. The diseases of the biliary system arise up mainly at children in age 5-6 and 9-12 years. A characteristic «chainlet» is traced: the anomalies of development and position of gall- bladder are dyskinesia - cholecystocholangitis is cholelythiasis, which start in child's age and will be to a full degree realized at adults.

2. The specific aims: - To define etiological and pathogenic factors of most widely spread functional diseases (dyskinesia of bile-excreting ways (DBEW)) and organic diseases of the biliary system at children. - To classify and analyze typical clinical picture of most widely spread functional diseases and organic diseases (acute and chronic , cholecystocholangitis, cholelythi- asis of the biliary system at children. - To make the plan of investigation and analyze data of laboratory and instrumental inves- tigations in typical course more widely spread functional diseases and organic diseases of the biliary system at children: general clinical analysis of blood and urine, biochemical blood tests, coprogramma, examination of intestinum’s microflora, ultrasound examina- tions of internal organs, fibroesophagogastro-duodenoscopy, x-ray examinations. - To demonstrate abilities of principles of treatment, reabilitation and preventive treatment of functional diseases and organic diseases of the biliary system at children. - To make the prognosis of life in functional diseases and organic diseases of the biliary system at children. - To demonstrate abilities of moral-deontological principles of medical specialist and prin- ciple of seniority in pediatrics.

3. Basic knowledge, skills, habits necessary for studing of the topic (inter-disciplinary inte- gration) The names of the preced- The acquired skills ing disciplines Normal anatomy To know anatomic peculiarities of gastrointestinal and the biliary systems of children. Normal physiology To hold ideas of physiological processes, which occur in biliary systems in children. Pathological physiology To identify physiological processes, which occur in digestive and the biliary systems on basis of revealed symptoms. Care of patients and nurse To demonstrate abilities and skills in care of infants with functional and or- practice ganic diseases of the biliary system and give preclinical medical aid. Propedeutics pediatrics To possess knowledge of basic clinical symptoms of functional and organic diseases of the biliary system; to have ideas of laboratory and instrumental methods of examinations. Domain of collection of anamnesis, clinical inspection of child, ability and skill of exposure and estimation of complexes of symptoms defeat of gastroduodenal highway skills, including pankreas, for children, domain knowledges about the additional methods of diagnostics of diseases of gastroduodenal system for children. Social medicine and organi- To use knowledge of the structure of medical aid to children for appropriate zation of health protection use of resources of health protection’s system in treatment and prophylaxis of occur of biliary systems. Pharmacology To possess knowledge of basic groups of medicine, which is used in treatment of biliary system.

4. The tasks for independent work during the training for the studies. 4.1. The list of basic terms, parameters, characteristics, which the student should master while training for the studies: № Term Determination 1. Dyskinesia of bile-excreting ways It is disorder of adjusting of secretion of bile, motility of gall- (DBEW) bladder and tonus of sphincteric apparatus as a result of uncoor- dinated, ill-timed, insufficient or surplus reduction of gall- bladder and/or sphincteric apparatus. 2. Acute cholecystitis Acute inflammation of gall-bladder. 3. Chronic cholecystitis Chronic recurrent inflammatory process of gall-bladder and in- (cholecystocholangitis) trahepatic bilious motions, that is accompanied by disirders of motility of bile-excreting ways and changes of physical and chemical composition of bile. 4. Cholelythiasis Inherited determined disease of liver and bile-excreting ways, based on disorder of processes of biligenesis and bile excretion.

4.2. The theoretical questions to the studies: 1. To define: dyskinesia of gall-bladder and sphyncteric apparatus. 2. Reason of dyskinesia of gall-bladder and sphyncteric apparatus. 3. Classification of dyskinesia of gall-bladder and sphyncteric apparatus. 4. Pathogenesis of dyskinesia of gall-bladder and sphyncteric apparatus. 5. Clinics and additional methods of diagnostics of dyskinesia of gall-bladder and sphync- teric apparatus. 6. Treatment, prophylaxis and prognosis of gall-bladder and sphyncteric apparatus.

4.3. The practical works (tasks) which are done at the studies: 1. Work with tests. 2. Work of students in wards near beds of children with dyskinesia of gall-bladder and sphyncteric apparatus, chronic cholecistitis (cholecistocholangitis) and cholelythiasis chronic pancreatitis. 3. Collection of complaints, history taking (life history, present history); a) Children’s objective investigation; b) Formulation of diagnosis according to modern classification; c) Composition of the plane sick child objective investigation; g) Composition of the plan of sick child treatment. 4. Clinical analysis of demonstrative case. 5. Salvation of situational tests.

5.The contents of the topic:

The theme’s content is thoroughly given in the following issues: lectures, methodological elaborations of the chair, teaching and scientific literature.

Functional biliary disorders Definition - is a disorder of the sphincter’ tonus and kinetics of the gall-bladder and bile ducts. Biliary dyskinesia is a symptomatic functional disorder of the gallbladder whose precise etiology is unknown. It may be due to metabolic disorders that affect the motility of the GI tract, including the gallbladder, or to a primary alteration in the motility of the gallbladder itself. Bili- ary dyskinesia presents with a symptom complex that is similar to those with biliary colic: 1. Episodes of right upper quadrant pain; 2. Severe pain that limits activities of daily living; 3. Nausea associated with episodes of pain; The presumed mechanism for biliary pain is obstruction leading to distension and inflamma- tion. This might result from incoordination between the gall bladder and either the cystic duct or the sphincter Oddi due to increased resistance or tone. Central projections from visceral noci- ceptors to the thalamus and cortex might lead to a more excitable state with hyperalgesia (severe pain evoked by mildly painful stimuli). Persistent central excitability might then result in allodynia where innocuous stimuli produce pain. Diagnosis. In order to diagnose biliary dyskinesia, the patient should have right upper quadrant pains similar to biliary colic but have a normal ultrasound examination of the gallbladder (no stones, sludge, microlithiasis, gallbladder wall thickening or common bile duct dilation). CLINICAL EVALUATION Diagnostic criteria: Must include episodes of pain located in the epigastrium and/or right upper quadrant and all the following: 1. Episodes lasting 30 minutes or longer; 2. Recurrent symptoms occurring at different intervals (not daily); 3. The pain builds up to a steady level; 4. The pain is moderate to severe enough to interrupt the patient’s daily activities or lead to an emergency department visit; 5. The pain is not relieved by bowel movements; 6. The pain is not relieved by postural change; 7. The pain is not relieved by antacids; 8. Exclusion of other structural disease that would explain the symptoms. Supportive criteria The pain may present with one or more of the following: 1. Associated with nausea and vomiting 2. Radiates to the back and/or right infra subscapular region 3. Awakens from sleep in the middle of the night Diagnostic criteria must include all the following: 1. Criteria for functional gallbladder and sphincter of Oddi disorder; 2. Gallbladder is present; 3. Normal liver enzymes, conjugated bilirubin, and amylase/lipase Diagnostic criteria must include both of the following: 1. Criteria for functional gallbladder and sphincter of Oddi disorder normal amylase/lipase Supportive criterion Elevated serum transaminases, alkaline phosphatase, or conjugated bilirubin temporarily related to at least two pain episodes Diagnostic criteria Must include both of the following: Criteria for functional gallbladder and sphincter of Oddi disorder and elevated amylase/lipase Classification hypertonic-hyperkinetic dyskinesia; hypotonic-hypokinetic dyskinesia. Clinical manifestation of hypertonic-hyperkinetic dyskinesia Duration of the disease up to 1 yr.; Pain syndrome; Dyspeptic syndrome; Manifestations of vegetative dysfunction, neurotic symptoms. Clinical manifestation of hypotonic-hypokinetic dyskinesia Pain syndrome; Dyspeptic syndrome; Hepatomegaly; Gallbladder symptoms are positive. Plan of examination Fool blood count; Biochemical test of blood; Serum aminotransferase; Serum bilirubin (predominantly the direct reacting fraction); Serum alkaline phosphatase; Albumin and globulin level; Stool test USE of the abdominal cavity + cholekynetics for functional investigations; Laboratory Tests of liver biochemistries and pancreatic enzymes must be normal. The following tests are necessary to eliminate calculous biliary disease, which can produce similar symptoms. Ultrasonography Transabdominal ultrasonography of the upper abdomen is mandatory. The biliary tract and pan- creas should be normal and gallstones or sludge absent. Ultrasonography readily detects stones equal to or greater than 3–5 mm in diameter or biliary sludge within the gall bladder, but it has a low sensitivity for smaller stones or biliary microcrystals. It also has a low yield for stones with- in the common bile duct. Endoscopic ultrasonography seems to be more sensitive than traditional transabdominal ultrasonography in detecting microlithiasis (tiny stones <3 mm) and sludge with- in the biliary tract, but the recommendation for its inclusion in standard workups requires further evaluation. Microscopic bile examination This procedure is necessary to exclude microlithiasis as a cause. Gall bladder bile can be ob- tained directly at the time of endoscopic retrograde cholangiopancreato-graphy (ERCP) or by aspiration from the duodenum following stimulation (e.g., cholecystokinin (CCK)-8 5 ng/kg i.v. over 10 minutes, or 50 ml MgSO4 instilled into the duodenum). Two types of deposits may be evident: (1) cholesterol microcrystals, which are birefringent and rhomboid shaped, best visual- ized by polarizing microscopy. Their presence provides a high diagnostic accuracy for micro- lithiasis; and (2) bilirubinate granules, which appear as red-brown deposits under conventional light microscopy. Endoscopy In the presence of normal laboratory and ultrasonographic findings, endoscopy is usually indi- cated to exclude upper gastrointestinal diseases. Tests for gall bladder dysfunction CCK–cholescintigraphy assessment of gall bladder emptying. This study continuously monitors the hepatic excretion of a radiopharmaceutical into the gall bladder and duodenum, using com- puter assistance to quantitate changes in radioactivity over the gall bladder. Filling of the gall bladder with radionuclide indicates patency of the cystic duct. Gall bladder emptying is ex- pressed as the gall bladder ejection fraction, the percentage decrease in net gall bladder counts following CCK infusion (CCK-8 slowly infused at 20 ng/kg over 30 minutes). Reduced empty- ing, which defines gall bladder dysfunction, can arise from either depressed gall bladder contrac- tion or increased resistance such as elevated tone in the sphincter Oddi. Furthermore, several other conditions that do not necessarily present with biliary colic can be associated with reduced gall bladder emptying. These range from intrinsic gall bladder disease (stones, cholecystitis) to neural and metabolic disorders, drugs, and even the irritable bowel syndrome. Although biliary- type pain is rarely elicited, the test appears to be a marker of this biliary disorder, based on evi- dence of the beneficial effect of cholecystectomy. Transabdominal ultrasonography This test measures gall bladder volume, which if followed serially after a stimulus (meal or CCK), reflects emptying. The technique is operator dependent and the results may not be repro- ducible in different centers. Ultrasonographic assessment of gall bladder emptying is currently not the standard for gall bladder dysfunction. Diagnostic workup Biliary tract symptoms should be evaluated by liver biochemistry, pancreatic enzymes, and ultra- sound examination of the abdomen. As a general recommendation we suggest that invasive inves- tigations should be withheld in those patients in whom episodes are infrequent and not accompa- nied by increased liver function tests. - If no abnormal findings are detected, CCK–cholescintigraphy should be used to assess gall bladder emptying. Abnormal gall bladder emptying (<40% ejection) indicates gall bladder dys- function. - If there is no obvious cause for impaired emptying, cholecystectomy is appropriate treatment. - If gall bladder emptying is normal, bile for microscopic examination to detect cholesterol microcrys- tals and bilirubinate can be obtained by duodenal drainage, at the time of gastrointestinal endoscopy or during ERCP. Magnetic resonance cholangiography or endoscopic ultrasound, where available, can be performed to detect lithiasis. Treatment of hypertonic-hyperkinetic dyskinesia 1.Diet; 2. Spasmolitics: platyphyllini hydrotartratis (amp. 0.2 % 1 ml); papaverini hydrochlorid- um (tab. 0.01, amp. 2 % 2 ml); no-spa (tab. 0.04 or amp. 2 % 2 ml) 3. Choleretic: cholagon, allocholum, cholenzynum, galstena, hepabene. Treatment of hypotonic-hypokinetic dyskinesia 1. Diet N 5 2.Prokinetic: motilium, domperidone (tabl. 0.01 g) 1 mg/kg/day 3.Choleretic and cholekinetic drugs: chophytol Herbal remedies for biliary dyskinesia are using plants that are producing the contraction and relaxation of gallbladder and are regulating the liver secretion of bile. There are a number of plants with choleretic action and cholagogue effect (contributing to the contraction of gallbladder and evacuation of bile into the duodenum). Artichokes – from this plant, with a good colecistokinetic effect, can be made teas. How- ever, the taste is quite bitter, which is why many people avoid to drink it. The resulting liquid should be consumed with small sips, unsweetened, in the morning on an empty stomach. Then, the patient should stay on the right side about 30 minutes for maximum effect of the tea. The al- ternative would be capsules with artichokes, which can taken half an hour before each meal. Yarrow – infusion may have a slightly sweet taste, according to some. Drink a cup of tea from this plant with a half hour before every meal to stimulate the evacuation of the bile. The infusion is prepared from a teaspoon of dried herb to a cup of boiling water. Cover the infusion and leave it untouched for 5 minutes. This is a very good tea for hyperacid gastritis, gastric and duodenal ulcer, hepatitis, abdominal bloating, nausea and loss of appetite. Dandelion – is one of the most effective herbs for liver drainage and evacuation of bile in the digestive tract. Represents an excellent way to stimulate liver activity. For a cup of infusion will be use 2 teaspoons of herb. Drink 2-3 cups a day. You can follow a diet with fresh dandelion stems (10-15 strains per day) if you have hepatitis or biliary dyskinesia. Other recommendations: Because biliary dyskinesia can be triggered by the nervous system, try to be calm, at least until the pain relieves. Take regular 4-5 meals per day and avoid foods with high level of fats. Avoid fried foods, sauces and stews; prefer boiled or baked foods. For proper disposal of the bile, is indicated that after meals to lie on the right side for 30 minutes.

Cholecystitis is an inflammation of the gallbladder, a small sack-like organ located in the upper right-hand side of the abdomen. In a healthy individual, the gallbladder temporarily stores bile, a substance produced by the liver that helps the body digest fat. After a meal, particularly a high-fat meal, bile passes from the gallbladder through the cystic and common bile ducts and into the small intestine where it helps process fats. Cholecystitis, which has long been considered an adult disease, is quickly gaining recogni- tion in pediatric practice because of the significant documented increase in nonhemolytic cases over the last 20 years. Gallbladder disease is common throughout the adult population, affecting as many as 25 million Americans and resulting in 500,000-700,000 cholecystectomies per year. Although gallbladder disease is much more rare in children, with 1.3 pediatric cases occurring per every 1000 adult cases, pediatric patients undergo 4% of all cholecystectomies. In addition, acalculous cholecys- titis, uncommon in adults, is not that unusual in children with cholecystitis. Because of the increasing incidence of gallstones and the disproportionate need for surgery in the pediatric population, consider cholecystitis and other gallbladder diseases in the differential diagnosis in any pediatric patient with or abdominal pain in the right upper quadrant, particularly if the child has a history of he- molysis. Pathophysiology: Cholecystitis is defined as inflammation of the gallbladder and is tradi- tionally divided into acute and chronic subtypes. These subtypes are considered to be 2 separate dis- ease states; however, evidence suggests that the 2 conditions are closely related, especially in the pe- diatric population. Most gallbladders that are removed for acute cholecystitis show evidence of chronic inflammation, supporting the concept that acute cholecystitis may actually be an exacerba- tion of chronic distension and tissue damage. Cholecystitis may also be considered calculous or acal- culous, but the inflammatory process remains the same. Chronic cholecystitis is most often related to gallstone disease but has been documented without gallstones. Its course may be insidious or involve several acute episodes of obstruction. The initiating factor is thought to be the supersaturation of bile, often with cholesterol crystals and/or cal- cium bilirubinate, which contributes to stone formation and inflammation. These processes lead to chronic obstruction, decreased contractile function, and biliary stasis, which contribute to further in- flammation of the gallbladder wall. Biliary stasis also permits the increased growth of bacteria, usual- ly Escherichia coli and enterococci, which may irritate the mucosa and increase inflammatory re- sponse. Chronic acalculous cholecystitis is less understood, but it may result from a functional defi- ciency of the gallbladder, which leads to spasm and an inability to appropriately empty its contents, causing chronic bile stasis. Physical: The physical examination in acute cholecystitis usually reveals right upper quad- rant tenderness. The classic triad is right upper quadrant pain, fever, and leukocytosis. The patient may have and a positive Murphy sign, ie, arrest of inspiration on deep palpation of the gallbladder in the right upper quadrant of the abdomen. Omental adherence to the inflamed gallbladder combined with distension may create a palpable mass between the 9th and 10th costal cartilages. The ductal system may become inflamed, causing cholangitis. In 50% of these cases, the examiner may find a Charcot triad. Charcot triad: This combination of right upper quadrant pain, fever, and jaundice is indica- tive of obstruction to the common bile duct and the presence of acute cholangitis. The Charcot triad is considered a medical emergency, and patients require immediate intervention. Biliary colic versus chronic cholecystitis: Performing a physical examination may be the only way to distinguish biliary colic from chronic cholecystitis. In chronic cholecystitis, the patient usually complains of tenderness to palpation in the right upper quadrant; however, the differentiation may be trivial given the high likelihood of chronic cholecystitis in the presence of recurring biliary colic. Lab Studies: In assessing for cholecystitis, appropriate laboratory studies include a CBC, gamma- glutamyltransferase (GGT) assessment, amylase measurement, urinalysis, direct and indirect biliru- bin tests, alkaline phosphatase measurement, and transaminase levels. In acute cholecystitis, the WBC count is elevated, with a predominance of polymorphonuclear cells and bands. Bilirubin, alka- line phosphatase and GGT levels rise secondary to a blocked biliary system. The traditional choles- tatic picture involves direct hyperbilirubinemia, with a direct-to-indirect ratio approaching 1:1. Am- ylase may be elevated even in the absence of obstructive pancreatitis. In addition, transaminases may show mild elevation but not a significant increase, unless obstruction has been severe enough to cause hepatocyte damage. Transaminase levels are more likely to rise early in patients with obstruc- tion of the common bile duct. Imaging Studies: Plain abdominal radiography may be used for initial screening in abdominal pain. Calcifica- tions representing radiopaque gallstones may be observed in the gallbladder or ductal system. Radio- paque gallstones contain more calcium bilirubinate and are more common in the pediatric population, especially in infants and children. In addition, complications such as porcelain gallbladder and emphy- sematous cholecystitis may be visible on radiographs, although these complications are rare in children. Abdominal ultrasonography has become the diagnostic tool of choice in evaluating choleli- thiasis. The accuracy of abdominal ultrasonography in depicting gallstones is estimated to be more than 95%, but its reliability in the accurate diagnosis of acute cholecystitis is more limited. Ultraso- nographic findings in acute cholecystitis include a discrete echodensity representing the gallstone, the presence of sludge, and, possibly, ductal anomalies or dilation. The gallbladder may be dilated with thickened walls. Imhof et al found gallbladder wall thickness of more than 3.5 mm to be a reliable independent diagnostic indicator of cholecystitis. Gallstones are often in a dependent position in the gallbladder and may move as the patient changes position. The reliability of ultrasonography is well established with both opaque and lucent gallstones. Results are immediate, and accessibility is usual- ly excellent. Oral cystography has been used in the past, but is now largely ignored because of the refine- ment of ultrasonography. Oral cystography involves the ingestion of contrast material that is secreted in the bile. Lack of visualization of the gallbladder indicates cholelithiasis. This procedure is limited by liver dysfunction and malabsorption. In addition, the contrast tablets have been associated with emesis and diarrhea, further complicating effectiveness. The most accurate tool in the diagnosis of acute cholecystitis is biliary scintography, other- wise known as the hepatic 2,6-dimethyliminodiacetic acid or hepatoiminodiacetic acid (HIDA) scan- ning. This procedure involves the intravenous injection of substances labeled with technetium 99m, taken into the hepatocytes, and excreted into the biliary system. Normal hepatic uptake without gallbladder visualization is diagnostic, but false positive results occur with decreased biliary function secondary to prolonged fasting and the use of hyperalimentation. Morphine augmentation of this test has been shown to decrease false positive results. Induced spasm of the sphincter of Oddi increases biliary pressure and enhances gallbladder filling. This test may be unnecessary, however, because the clinical diagnosis and treatment are determined by the symptoms and presence of gallstones or sludge. Ultrasonography has proved its usefulness in depicting gallstones, does not rely on contrast, and, therefore, may be safer. Other imaging techniques that can be used in the diagnosis of cholecystitis include MRI and CT, especially in cases in which ultrasonography is not helpful. Ultrasonographic results may be compromised by ileus, surgical incisions, and coexisting diseases, especially those found in patients who are critically ill. MRI and CT may be more sensitive than ultrasonography in detecting inflam- mation within and around the gallbladder. In addition, the presence of other sources of abdominal sepsis are more easily discovered and treated by means of MRI and CT. Other Tests: Other tests associated with the diagnosis and treatment of cholecystitis include cholecystokin- in (CCK) stimulation, intraoperative cholangiography, and endoscopic retrograde cholangiopancrea- tography (ERCP). CCK stimulation may be employed during other imaging studies, such as cholesc- intigraphy. Gallbladder dyskinesia after CCK administration is diagnostic of gallbladder hypofunc- tion and may be useful in discerning acalculous or chronic cholecystitis and acute inflammation. Intraoperative cholangiography, whether intravenous or percutaneous, is widely used for the visualization of the gallbladder and ductal system. However, cholangiography can be time- consuming and an added expense to the patient, although some data show no statistical difference in operative time with and without its use. Consider cholangiography for any risk of obstruction of the common bile duct. Indications are a history of jaundice, pancreatitis, dilated common bile duct, and the presence of small gallstones. The benefits of using cholangiography have not been proven for routine cholecystectomy, routine screening for congenital anomalies, or assessment of the common bile duct for obstruction in the absence of clinical suspicion.

6. The materials for self-control A. Sample case report

1. Girl 12 years, present problems on dull pain in the stomach region, aching character, which arise up in 30-45 minutes after the meal intake, and also weakness, fatigue, frequent head- ache. First above mentioned complaints appeared 6 months ago, but inspection and treatment was not. Mother - 40 years, suffers by ulcerous illness of duodenum. Father - 42 years, chronic gastroduodenyt. Objectively: growth 137 sm, mass 31 kg. Skin is pale, moderate humidity. An abdomen is not enlarged. At superficial and deep palpation tension of muscles and soreness, and also pain in epigastrium is determined in right hypochondrium. A liver +1,5 sm, the border of liver is soft, elastic, painless. The Ortner-Grekov symptom (+). From the side of lungs and heart - without pathology. A stool is regular, shaped, sometimes light. General blood test: Hb - 130 g/L, CI - 0,93, red corpuscles of 4,6Х1012/L, leucocytes - 7,Ох 109/L: s - 2%, s - 66%, е - 2%, l - 25%, m - 5%, ESR 7 mm/h. General urine test: in a norm. Biochemical blood test: general albumen - 72 г/л, ALT - 19 IU, AST-24 IU, AP - 138 IU (norm 70-140), amilase - 100 IU (norm 0-120), timolov test - 4 IU, total billirubin – 15 mkm/L, direct - 3 mkm/L. USI of organs of abdominal region: a liver is contours even, parenchyma is homogene- ous, echogenic is increased, vessels are not extended, a portal vein is not changed. Gall-bladder 85х37 mm (norm 75х30), walls are not thickened. Choledochus - to 3,5 mm (norm 4), walls is not thickened. After bile-excreting breakfast is a gall-bladder grew short on 10%. 1. Create the main diagnosis. 2. Create the plan of general and additional inspection. 3. Create a plan of treatment. 4.Write out to this child of allochol.

2. Boy 7 years, entered permanent establishment in order of the centre system health be- fore the going in school. The half-year disturb stomach-aches ongoing. With the intake of food is not relate, frequent in opinion of mother pain arises up after the emotional overloads - much hur- ries outsides, plays hockey. In anamnesis is coli-infection in 5 months, dysentery - in 3 years, is often ill the respirator diseases. During 6 months three times inspected in the presence of eggs of helmints in an excrement is result negative. Objectively: normal body structure, satisfactory feed. Skin, visible mucus are clean. Or- gans of thorax without pathology. The abdomen of the normal form, during palpation is soft, is determined small painful around umbilicus and in right hypochondrium. A liver palpate on the edge of costal arc. There are the signs of vegetodystony - sweats easily, proof, red dermogra- phism, emotional lability. A EPN -doctor diagnosed chronic tonsillitis. 1. Create the main diagnosis. 2. Create the plan of general and additional inspection. 3. Create a plan of treatment. 4.Write out to this child of cholosas.

3. A boy, 13 years, entered the gastroenterology unit with complaints on a stomach-ache, which localized in epygastrium and arise up in 15-20 minutes after the intake of meal and dura- tion from 30 minutes to a few hours; decrease of appetite, nausea, bitter taste in to the mouth. He is ill during 2 years, when the complaints listed above appeared first, however treatment was not performed. 2 weeks prior to the receipt in permanent establishment an abdominal syndrome in- creased at a boy, that served by the reason of hospitalization. In 8 years a child carried an intestinal infection. Feeds irregularly, likes smoked food, rich food fried, and cakes. Mother 40 years, suffers on chronic cholecistitis. Father - 42 years, is ill ulcerous disease of duodenum. A grandmother (on a mother) has cholelithiasis . Objectively: on the skin of thorax and back are single elements as vascular asterisks. An abdomen is not enlarged, painful in epygastrium at deep palpation. A liver comes forward from under the edge of costal soft, elastic, slightly painful. The Ortner symptom (+). From the side of other organs - without pathology. General blood test : Hb - 140 g/L , CI - 0,93, red corpuscles - 4,5х1012/L, leucocytes - 9,5х 109/L: s - 2%, s - 64%, е - 1%, l - 26%, m - 7%, ESR 15 mm/h. General urine test : in a norm. Biochemical blood test: general albumen - 75 g/L , ALT - 18 IU, AST-25 IU, AP - 136 IU (norm 70-140), amylasa - 100 IU (norm 0-120), timolov test - 4 IU, total billirubin – 15 mkm/L, direct - 3 mkm/L. FEGDS: the mucous membrane of esophagus is red, cardia closes up. Mucous membrane of stomach in the antral region is hyperemic, bulb of middle sizes, was swollen, hyperemia. The Postbulbar partts are not changed. USI of organs of abdominal region: a liver is contours even, parenchyma is homogene- ous, echogenic is not increased, vessels are not extended, a portal vein is not changed. Gall- bladder of ordinary form, walls - 5-6 mm (norm 2 mm), expressed stratified of walls, internal contours are uneven. Choledochus - to 5,5 mm (norm 4), walls is thickened. 1. Create the main diagnosis. 2. Create the plan of general and additional inspection. 3. Create a plan of treatment. 4. Write out to this child of chophytol.

4. A child is 7 years. Complaints about a stomach-ache attack character, which arises up after the psychical loading, use of cold drinks, ice-cream. There is the state of child of middle weight at examination. A skin and visible mucous membranes is pale. The nasal breathing is free. Pharynx is not hyperemic. Above lungs there is the vesicular breathing. The heart sounds are clean, rhythmic. An abdomen is not enlarged, painful in right hypochondrium during deep palpation. Physiological functions in a norm. General blood test : Hb - 118 g/L, Er-3,7х10/L, CI - 1,0, L-4,6х10/L, ESR 14 mm/h. General urine test : in a norm. The duodenal probe exposed prolongation of 1-and, 2-and and 3-and phases of factious research with diminishment of volume of bile in 1 and 2 phases. 1. Create the main diagnosis. 2. Create the plan of general and additional inspection. 3. Create a plan of treatment. 4. Write out to this child of dyuspatalyn.

5. Gastroenterology unit of child's hospital a boy 13 years with complaints about frequent attack pain in right hypochondrium and near a belly-button entered, which arises up after the physical loading during the last 3 months, propensity to constipations. During the objective in- spection: skin of normal color, at superficial palpation a stomach is soft, painful in right hypo- chondrium. The lower edge of liver at the level of costal arc, an edge is pungent, is painless. Physiology functions in a norm. GBT: Hb-128 g/L, Er-3,6*1012/L, CI - 1,0, L-5,4*109/L, ERS - 7 mm/h. GUT: urines is pathology is not exposed. An excrement on eggs is an intestinal worm, enterobiosis scrape are negative. During the duodenal probe portion 1 was not took, portion 2 - without the changes. 1. Create the main diagnosis. 2. Create the plan of general and additional inspection. 3. Create a plan of treatment. 4. Write out to this child of gimekromon (Odeston).

B. Tasks for self-control

1. Child of 8 years, complains of colicky sharp pain in the right hypochondrium оп exercise. Skin and visible mucous tunics are of usual color. There is по pathology from the side of heart and lungs. The abdomen is soft, painless, the liver is not megascopic, the re is pain in Kehr's point. Ultrasonic scanning shows gall-bladder without specific features, at the use of а prokinetic the gall-bladder contracted оп 80%. Temperature of the body, blood and urine tests are in norm. What is your diagnosis? А. Gallbladder dyskinesia of а hyperkinetic type В. Gallbladder dyskinesia of а hypokinetic type С. Appendicitis D. Chronic cholecystitis Е. Chronic pancreatitis

2. 10-year-old girl has for а year colicky pains in the right hypochondrium after the emotional and physical overstrain. She is irritable, gets tired easily. At the examination general analysis of blood is in norm, the ultrasound scanning shows по concrements in the gall-bladder. Duodenal intubation provoked pain attack, bile was not received. Biliary dyskinesia of а hypertonic- hyperkinetic type was diagnosed. Choose the optimum therapy regimen. А. Antibiotics, cholekinetics В. Cholekinetics, purgatives С. Vitamins, duodenal tubages D. Antibiotics, mineral waters Е. Sedative preparations, spasmolysants, thermal procedures

3. А child is 7 years old. It complaints of colicky abdominal pains, which arises оп physical overstrain, after intake of cold drinks, ice-cream. After the clinicinstrumental examination the diagnosis is: gallbladder dyskinesia of а hyperkinetic type What preparations are to bе prescribed first of аll for the treatment? А. Choleretics and cholekinetics В. Sedative ones and cholekinetics С. Spasmolysants and choleretics D. Antioxidants Е. Antibiotics

4. At ultrasound scanning of contractive ability of а gall-bladder of а bоу of 11 years it was found out that the diameter of gall-bladder contracted less than оn оnе half and gall-bladder movement function rate (GМFR) is 0,7. It would bе optimum to prescribed to this child. А. Choleretics and cholekinetics В. Choleretics С. Cholekinetics D. Spasmolysants Е. Nothing of аbоvе mentioned

5. А girl of 5 years was diagnosed with gallbladder dyskinesia of а hypotonic type. What from transferred beside below the purpose to plug in complex therapy? А. Physiotherapy of restorative type В. Cholekinetics С. Duodenal intubation D. Spasmolytic preparations Е. Mineral water of high mineralization

6. А bоу of 12 years оn а background of the satisfactory condition, normal sizes of а liver and а spleen, has icterus of skin and scleras. In the blood test there are; alanine aminotransfcrase - 0,6 mmole/l, total bilirubin - 51 mmole/l, conjugated bilirubin - 1 О mcmole/l, unconjugated biliru- bin - 41 mcmole/l, Hb - 125 g/l, red corpuscles - 4,1 х 10* 12g/l. What disease is it possible to think about first of аll? А. Gilbert's syndrome В. Viral hepatitis С. Hemolytic anemia D. Toxic hepatitis Е. Biliary dyskinesia

7. In forming of chronic cholecystitis the greatest role is played bу: А. Bacterial and viral infections В. Pathogenic fungi С. Helminths D. Protozoa Е. Stress.

8. Stage ofthe cholelithiasis which is most typical for hild's age: А. Physical and chemical ones В. Surgical one С. Latent оnе D. Clinical one Е. Easy оnе

9. Among the anomalies of the development and position of the gall-bladder prevail: А. Anomalies of quantity or structure В. Anomalies of position С. Anomalies of sizes D. Anomalies of form.

10. Clinical syndrome which is least typical for the chronic cholecystocholangitis: А. Pain В. Chronic constipation С. Dyspepsia D. Chronic intoxication Е. Asthenovegetative syndrome. 7. Reference literature Basic: 1. Nelson textbook 20th Edition by Robert M. Kliegman, MD, Richard E. Behrman, MD, Hal B. Jenson, MD and Bonita F. Stanton, MD. Видавництво: SAUNDERS 2015; 2. Rogers’ textbook of Pediatric Intensiv Care Edition by David G.Nichols et all. Ви- давництво: United Nations Children's Fund, 2015, New York. 3. CURRENT Diagnosis & Treatment Pediatrics 23th Edited by William W. Hay, Jr., Robin R. Detrding, et al. McGraw-Hill Education., Видавництво: The McGraw-Hill Companies 2016.

Additional 1. Kapitan T. V. Preliminary study of children diseases with child care. Moscow: Medpress-inform, 2014.

The methodical instructions are compiled by Bubyr L.N.

Methodical instructions were discussed and approved at the meeting of department of Pediatrics №2 protocol № ___ of ______20____.

Additions and changes were made “____” ______20____ Additions and changes were made “____” ______20____ Additions and changes were made “____” ______20____ Additions and changes were made “____” ______20____ Additions and changes were made “____” ______20____ MINISTRY OF PUBLIC HEALTH OF UKRAINE UKRAINIAN MEDICAL STOMATOLOGICAL ACADEMY DEPARTMENT OF PEDIATRICS №2

Approved at the meeting of department of pediatrics №2 Minutes № 21 " 05 " 06 2020 Head of the department PhD, Prof. Kryuchko T.O. ______

METHODICAL INSTRUCTIONS FOR INDEPENDENT WORK OF STUDENTS DURING THE TRAINING FOR THE PRACTICAL (SEMINARS) CLASS

Academic discipline Pediatrics Моdule № 1 Substantial mоdule № 3 Topic of lesson Functional and organic diseases of bowels in children.

Course 4 Faculty medical

Poltava 2020

1. 1. Actuality of topic: More than one third of children complain of abdominal pain lasting two weeks or longer. The diagnostic approach to abdominal pain in children relies heavily on the history provided by the parent and child to direct a step-wise approach to investigation. If the history and physical examination suggest functional abdominal pain, constipation or peptic disease, the response to an empiric course of medical management is of greater value than multiple "exclusionary" investigations. A symptom diary allows the child to play an active role in the diagnostic process. The medical management of constipation, peptic disease and inflammatory bowel disease involves nutritional strategies, pharmacologic intervention and behavior and psychologic support. 2. The specific aims: - To define the etiologic and pathogenetic factors of functional and organic diseases of intestine at children: bowel irritation syndrome, functional constipation, unspecific ulcerous colitis, Crohn disease. - To classify and analyze typical clinical picture of most widely spread of functional and organic diseases of intestine at children: bowel irritation syndrome, functional constipation, unspecific ulcerous colitis, Crohn disease. - To make the plan of investigation and analyze data of laboratory and instrumental investigations in typical course more widely spread of functional and organic diseases of intestine at children: general clinical analysis of blood and urine, biochemical blood tests, coprogramma, examination of intestinum’s microflora, ultrasound examinations of internal organs, fibroesophagogastroduodenoscopy, x-ray examinations. - To demonstrate abilities of principles of treatment, reabilitation and preventive treatment of functional and organic diseases of intestine at children: bowel irritation syndrome, functional constipation, unspecific ulcerous colitis, Crohn disease. - To carry out differential diagnosis and set preliminary clinical diagnosis of functional and organic diseases of intestine at children: bowel irritation syndrome, functional constipation, unspecific ulcerous colitis, Crohn disease. - To make the prognosis of life of functional and organic diseases of intestine at children: bowel irritation syndrome, functional constipation, unspecific ulcerous colitis, Crohn disease. - To demonstrate abilities of moral-deontological principles of medical specialist and principle of seniority in pediatrics.

3. Basic knowledge, skills, habits necessary for studying the subject (interdisciplinary integration). Name of previous disciplines Acquired skills Anatomy, physiology Anatomical and physiological features of the digestive system in children. Characterize the performance standards of the digestive system in children Pathological anatomy and Know the specifics of the hormonal and neurological regulation of intestinal secretion, physiology digestion, morphological changes in the intestinal mucosa, the mechanism of formation of erosions, the mechanism of development of clinical signs. Interpretation of test results. Propedeutiks childhood Know the methods of investigation of the digestive system in children, instrumental diseases methods of examination of the state of the gastrointestinal tract in children. Spend palpation, percussion, auscultation of the digestive system of the child. Microbiology Know the procedure crops secretions and secrets and interpret the results. Pharmacology Knowing the group of drugs used for the treatment of intestinal diseases. Prescribe the necessary drugs, taking into account the state of the child's age and weight, the characteristics of the individual reactions. To be able to write prescriptions. Surgery Know clinicoanamnestic complex signs of complications of intestinal diseases. Fundamentals of surgical treatment. Faculty Pediatrics Clinical-anamnestic and laboratory-instrumental complex features intestinal diseases in children of different ages. Identify signs of organic and functional intestinal diseases in children of different ages. Intradisciplinary integration Give a clinical evaluation of changes in clinical, biochemical and immunological studies of blood serum, to interpret the data of instrumental methods of examination.

4. The tasks for independent work during the training for the studies. 4.1. The list of basic terms, parameters, characteristics, which the student should master while training for the studies:

Term Definition Functional bowel Are functional gastrointestinal disorders with symptoms attributable to the middle or lower disorders gastrointestinal tract. These include the IBS, functional bloating, functional constipation, functional diarrhea, and unspecified functional bowel disorder. Irritable Bowel Syndrome IBS is a functional bowel disorder in which abdominal pain or discomfort is associated with defecation or a change in bowel habit, and with features of disordered defecation. Functional Bloating Functional bloating is a recurrent sensation of abdominal distention that may or may not be associated with measurable distention, but is not part of another functional bowel or gastroduodenal disorder. Functional Constipation Functional constipation is a functional bowel disorder that presents as persistently difficult, 2 infrequent, or seemingly incomplete defecation, which do not meet IBS criteria. Functional diarrhea Functional diarrhea is a continuous or recurrent syndrome characterized by the passage of loose (mushy) or watery stools without abdominal pain or discomfort. Unspecified Functional Individual symptoms discussed in the previous sections are very common in the population. Bowel Disorder These occasionally lead to medical consultation, yet are unaccompanied by other symptoms that satisfy criteria for a syndrome. Such symptoms are best classified as unspecified. Chronic enteritis Polietiologic disease which is based on inflammatory and degenerative processes in the wall of the small intestine that causes a violation of the digestive, excretory and motor functions of the body and, as a consequence, a metabolic disorder. Chronic colitis (chronic Chronic inflammatory-dystrophic lesions of the mucous membrane of the colon with a colitis nonulcerated) predominance in the clinical signs of functional disorders. Ulcerative colitis Chronic disease with progressive relapsing course, with periods of bloody diarrhea, which is based on the inflammatory process in the colon with severe destructive changes of the mucous membrane and submucosal layer. Crohn's disease (syn.: Chronic, relapsing disease characterized by transmural granulomatous inflammation, terminal, regional ileitis, ulcerative mainly the terminal ileum and cecum. granulomatous enterocolitis)

4.2. The theoretical questions to the studies: 1. Current views on the etiology and pathogenesis of organic and functional disorders of functional and organic diseases of the bowel (irritable bowel syndrome, chronic enteritis, chronic colitis, ulcerative colitis, Crohn's disease) in children. 2. Classification (clinical, clinical and pathogenetic) organic and functional disorders functional and organic bowel disease in children. 3. Main clinical syndromes of organic and functional diseases of the functional and organic diseases of the bowel (irritable bowel syndrome, chronic enteritis, chronic colitis, ulcerative colitis, Crohn's disease) in children. 4. Differential diagnosis of organic and functional disorders of the functional and organic diseases of the bowel (irritable bowel syndrome, chronic enteritis, chronic colitis, ulcerative colitis, Crohn's disease) in children. 5. Laboratory and instrumental diagnosis of organic and functional disorders of the functional and organic bowel diseases (irritable bowel syndrome, chronic enteritis, chronic colitis, ulcerative colitis, Crohn's disease) in children. 6. Current approaches to the treatment of organic and functional disorders of the functional and organic bowel diseases (irritable bowel syndrome, chronic enteritis, chronic colitis, ulcerative colitis, Crohn's disease) in children. 7. Principles of clinical supervision for children with organic and functional diseases of the gastrointestinal tract. 8. What is Functional bowel disorders? 9. Diagnostic Criteria for Irritable Bowel Syndrome? 10. What is Functional Bloating? 11. Diagnostic Criteria for Constipation? 12. What is Functional diarrhea? 13. Diagnostic Criteria for Unspecified Functional Bowel Disorder? 14. What is the Bristol Stool Form Scale? 15. Five Components of the Evaluation of Children with Abdominal Pain. 16. Recurrent Abdominal Pain Syndrome.

4.3. The practical works (tasks) which are done at the studies: – To collect complaints, anamnesis of the disease. – To conduct an objective examination of the patient. – To assess results of laboratory and instrumental methods. – To make on a preliminary diagnosis. – To perform a differential diagnosis. – To assign treatment according to the protocol. – To make out a plan of rehabilitation. – To write out recipes.

5. The contents of the topic: Inflammatory Bowel Disease. Abdominal pain is frequently reported in children with ulcerative colitis and Crohn's disease. The pain, which typically occurs in the lower abdomen, is cramping in nature and increases after meals or activity. The pain is reduced by eating smaller meals, which contributes to the anorexia and growth impairment that occur in children with inflammatory bowel disease. The diagnosis is relatively easy when the child has bloody diarrhea, the need to defecate during the night, perianal disease or an ileal mass on abdominal examination. More subtle features include delayed puberty, anemia that is unresponsive to iron therapy, recurring oral aphthous ulcers, chronic liver disease, or large

3 joint synovitis or arthritis.18 The diagnosis is established by small bowel barium contrast x-ray and colonoscopy with biopsies. The management of inflammatory bowel disease in childhood is summarized in Table 3. Management of Inflammatory Bowel Disease in Children

Supportive care for child and family • Provide educational materials for child, parents, teachers • Give information about support groups for children and parents • Offer psychologic counseling for depression, denial and noncompliance • Expect reactive self-manipulation of medication dosages and diet Nutritional support • Correct deficits of macronutrients and micronutrients • Deliver 125 percent of calories for height age • Recommend routine multivitamin and mineral supplements • Discourage "quick cure" diets and fads • Administer intravenous nutrition to patients with intractable Crohn's disease or fistula and before surgery • Consider consumption of an elemental diet as primary therapy in patients with small bowel Crohn's disease Anti-inflammatory/immunomodulatory medication • Prednisone (oral, intravenous, topical enema) --Valuable in all forms, but use must be balanced against side effects --Useful as chronic alternate-day therapy in adolescent patients with Crohn's disease • Salicylates: sulfasalazine (Azulfidine), mesalamine (Asacol, Pentasa, Rowasa), aminosalycylic acid (Paser Granules) --Valuable in treating mild to moderate colitis • Metronidazole (Flagyl; possibly ciprofloxacin [Cipro] as well in older children) --Useful in treating Crohn's perianal or fistula disease --Also useful in treatment of complicating Clostridium dificile infection • Azathioprine (Imuran)/6-mercaptopurine (Purinethol) --Valuable in treating moderate to severe Crohn's colitis, ulcerative colitis • Fish oil (EPA, Sea Omega, Promega) --Valuable in treating mild ulcerative colitis Surgical resection • Total is curative in cases of ulcerative colitis • Useful in cases of toxic , and dysplasia in patients with ulcerative colitis • Useful in treating Crohn's obstruction, fistula, abscess • Useful when medical therapy fails or side effects of medication are intolerable Final Comment. Once the etiology of chronic abdominal pain is established, the process of patient and family education has just begun. Careful follow-up is necessary to monitor compliance with treatment, restoration of normal activities and appropriate family interventions. Children do not like to feel "different," and they often resist the need for long-term nutritional or pharmacologic intervention. Growth parameters must be followed carefully. Support groups for the family and the child can be invaluable. Most importantly, the child must feel that the family physician understands that the pain is real, that the child's input is as valuable as the parents' and that information shared in confidence will be kept confidential if at all possible. Cystic fibrosis is the most common lethal genetic disease and the most common cause of malabsorption among white American or European children. By the end of the 1st yr of life, 85–90% of children with cystic fibrosis (CF) have pancreatic insufficiency, which, if untreated, can lead to malnutrition. Treatment of the associated pancreatic insufficiency leads to improvement in absorption, better growth, and normalized stools. Pancreatic function can be monitored in children with CF with serial measurements of fecal elastase. Certain mutations in the cystic fibrosis gene have been associated with idiopathic chronic pancreatitis. Shwachman-Diamond Syndrome (SDS) is an autosomal recessive syndrome (1/20,000 births), consisting of pancreatic insufficiency; neutropenia, which may be cyclic; neutrophil chemotaxis defects; metaphyseal dysostosis; failure to thrive; and short stature. Some patients with SDS have liver or kidney involvement or learning difficulty. Patients typically present in infancy with poor growth and greasy, foul-smelling stools that are characteristic of malabsorption. These children can be readily differentiated from those with cystic fibrosis by their normal sweat chloride levels, lack of the cystic fibrosis gene, characteristic metaphyseal lesions, and fatty pancreas on CT examination. Despite adequate pancreatic replacement therapy, poor growth frequently continues. Pancreatic insufficiency is often transient, and steatorrhea may spontaneously improve with age. Recurrent pyogenic infections (otitis media, pneumonia, osteomyelitis, dermatitis, sepsis) are common and are a frequent cause of death. Thrombocytopenia is found in 70% of patients and anemia in 50%. Development of a myelodysplastic syndrome may occur and transformation to acute myeloid leukemia has been reported in up to 33% and 24% of patients, respectively. The gene for SDS is found on chromosome 7. Pathologically, the pancreatic acini are replaced by fat with little fibrosis. Islet cells and ducts are normal. The fatty pancreas has a characteristic hypodense appearance on CT and MRI scans. Pearson Syndrome. This is a sporadic mitochondrial DNA mutation affecting oxidative phosphorylation that manifests in infants with severe macrocytic anemia and variable thrombocytopenia. The bone marrow demonstrates vacuoles in erythroid and myeloid precursors as well as ringed sideroblasts. In addition to its role in severe bone marrow 4 failure, pancreatic insufficiency contributes to growth failure. Other mitochondrial DNA mutations are associated with the development of diabetes mellitus (Kearns-Sayre, chronic progressive external ophthalmoplegia, diabetes with deafness syndromes). Mitochondrial DNA mutations are transmitted through maternal inheritance to both sexes or are sporadic. Isolated Enzyme Deficiencies. Isolated deficiencies of trypsinogen, enterokinase, lipase, and colipase have been reported. Although enterokinase is a brush border enzyme, deficiency causes pancreatic insufficiency because pancreatic proteases remain inactive. Deficiencies of trypsinogen or enterokinase manifest with failure to thrive, hypoproteinemia, and edema. Isolated amylase deficiency is typically developmental and resolves by age 2–3 yr. CHRONIC INTESTINAL DISEASES Bowel disease in children are extremely common, and their incidence is increasing, they differ variability and diversity of clinical symptoms. The structure of chronic bowel disease are distinguished: – functional (irritable bowel syndrome) – inflammation (chronic colitis, chronic enteritis) – destructive (ulcerative colitis, Crohn's disease). In addition, an important place, especially in young children sit hereditary bowel disease, the course is accompanied by a syndrome of malabsorption (celiac disease, mukoviscydosis, lack of disaccharidases, exudative enteropathy). In the formation of many diseases, not just bowel dysbiosis plays an important role. Despite the fact that chronic bowel disease more or less disrupted the activities of all its parts, it is advisable to provide disease mainly small and large intestines. IRRITABLE BOWEL SYNDROME Irritable Bowel Syndrome (IBS) (irritable syndrome, intestinal dyskinesia, dyskinesia of the colon, spastic colitis, spastic colon, functional kolopatiya, mucous colic, ulcers neurosis etc. ..) - 58 K code - functional gastrointestinal disorder caused by disorders motor function of the intestine characterized by abdominal pain and intestinal functions without violation of specific organic pathology. Etiopathogenesis In the formation of the IBS are important psychological and emotional factors, acute and chronic stress. Vegetative dystonia, neurosis states of both functional and organic nature naturally accompanied by changes in motor function of the intestine. Family feuds, illness or death of parents or close relatives of the child, parent alcoholism and drug addiction, conflicts at school or family often precede the appearance or progression of motor disorders of bowel function and related clinical symptoms. In the etiology of the disease is undeniable role of infectious factors, poor nutrition with decreasing amounts of foods rich in fiber, poor tolerance of some nutrients, food allergies, changes in the composition and activity of the intestinal microflora (dysbiosis). Kolodyskinesia often occurs against a background anomalies of the bowel (megacolon, dolihosyhma), diseases of the rectum (anal fissures, hemorrhoids), chronic inflammatory diseases of the digestive system (cholecystocholangitis, gastropancreatitis). Factors that contribute to the formation of IBS are sedentary, frequent use of , the presence of foci of chronic infection in the body, asthenic physique of the child, which is often combined with vistseroptosis. Various etiological factors are usually combined with each implemented with the participation of cholinergic and adrenergic nerve dysfunction which causes disorders of the autonomic nervous system. Due to the high sensitivity of receptors to the wall of the colon distention occurs overstimulation of spinal neurons with the formation of pain. Important role in the genesis of IBS also play hastrointestinal peptide hormones (gastrin and cholecystokinin), which stimulate intestinal motility, imbalance neurotransmitters (acetylcholine, epinephrine, norepinephrine), increased production of prostaglandins in the intestine.

Classification Classification of IBS based on Rome III criteria, 2010 In accordance with these recommendations, the SEC is defined as a set of functional (non-organic lesion of the intestine) disorders for more than 3 months, the main clinical manifestations of which are abdominal pain (usually disappears after a bowel movement ), which is accompanied by bowel function (constipation, diarrhea or alternating them), and flatulence, "rumbling" bowel, sensation of incomplete emptying or compelling urge to defecate. At the same time emphasize that most observations disturbed bowel function is not total, and its middle and distal, thus the term "irritable bowel syndrome" is more legitimate than the "irritable bowel syndrome", but it is still used much more frequently. Entitled "irritable bowel syndrome" this diagnosis is presented in the International Classification of Diseases (ICD-10) in section 58. Clinical classification of IBS is based on key clinical syndrome and involves the selection of these variants of the disease, given the prevalence of the main clinical symptoms: mainly of diarrhea; mainly from constipation, diarrhea, mixed variant + constipation, alternating varіant - alternating constipation and diarrhea. As diarrhea and kolostatychnyy options may be accompanied by abdominal pain and flatulence. 1. IBS – С (IBS with constipation) 2. IBS – Д (IBS with diarrhea) 3. IBS – М (mixt syndrome, constipation + diarrhea) 4. IBS – А (alternation constipation and diarrhea) Depending on the nature of intestinal motility identified two main forms of intestinal dyskinesia with IBS - hypomotorical and hypermotorical.

5 Children often find hypomotorical intestinal dyskinesia, accompanied mainly constipation. Less - dyskinesia accelerated motility (hyper), which occurs mainly functional diarrhea, the occurrence of which is particularly important role of psycho-emotional factors that help to reduce the absorption of water and electrolytes, increased secretion of mucus, intestinal juice and increased intestinal motility. Sometimes diarrhea can also occur hypomotorical dyskinesia, which is caused by hypersecretion enhanced: the liquid feces is not associated with increased peristalsis, and his secondary dilution. In turn, hyperdyskinesia may be associated with constipation; This is caused by increased bowel non-propulsion segmented movements that promotes mixing of gut contents, but hinders its progress. Symptoms of Irritable Bowel Syndrome in Children While every child is different, other common symptoms of IBS include: • A change in frequency of bowel movements • A change in the consistency of bowel movements • An improvement in pain level when a bowel movement occurs • While every child is different, other common symptoms of IBS include: • Diarrhea • Constipation • Mucus in the bowel movement • Abdominal bloating • Feeling as if the bowels are not emptied after a bowel movement • Nausea • Dizziness • Loss of appetite. The leading clinical symptom of IBS is a violation of feces, often in the form of constipation. Coprostasia localized predominantly in the descending and sigmoid colon. Constipation can be varied. Sometimes there are emptying daily, and children complain only difficulty in defecation and lack of feeling of bowel movement. Most irregular bowel movement every other day or once every 3-4 days, sometimes only after using or laxatives. Cal often dry, hard, often of the "sheep" and godlike or ribbon. Some patients note a heterogeneous calories: behind dense stands and even mushy liquid ("Shut diarrhea"). Despite the long and persistent constipation for a long time, children can not complain, later joined by abdominal pain, which is characterized by considerable polymorphism: from short-term, significant intensity to the long aching, which is localized more frequently in inguinal or umbilical area. At the same time there is a feeling of heaviness, fullness, bloating. Over the long course of the disease often show signs of intoxication: lethargy, increased fatigue, irritability. Much less IBS in children is accompanied by diarrhea, which is associated with both increased motor function (true diarrhea) and increased intestinal secretion (false diarrhea). In such a situation noted accelerated bowel movements with sparse, abundant emptying. Characterized by the appearance of diarrhea already in the early morning hours ("diarrhea- clock"). Sometimes diarrhea occurs only impermanent and during excitement, agitation ("Bear disease"); Sometimes children complain of frequent urge to defecate, which causes anxiety and may cause or fixing stressful situation and fear " ashamed". In pain and dyspeptic phenomena in IBS often noted headaches (like migraines), feeling of "lump" in swallowing, pain in the lumbar region, drowsiness, feeling trembling, urination disorder. These symptoms are often combined with signs of functional disorders of the stomach (dyspepsia inorganic) - a feeling of heaviness and fullness in the epigastric region, nausea, belching, due to common pathogenic mechanisms of these diseases, the essence of which is to initiate motility. An objective examination of patients with IBS pain on palpation of the abdomen can not be, however, often reveal tenderness along the colon, most pronounced in the projection of the transverse colon. Sometimes spasmodic palpable bowel loops. In the IBS, accompanied by diarrhea during palpation reveal vurchannya. In this note the discrepancy between the number and variety of complaints, no signs of disease progression and satisfactory typically, the general condition of the child. Important differential diagnostic symptoms are lack of reaction temperature, impurities of blood in the stool, maintaining body weight, normal levels of peripheral blood. Diagnosis Diagnostic criterion* Recurrent abdominal pain or discomfort at least 3 days/month in the last 3 months associated with two or more of the following: 1. Improvement with defecation; 2. Onset associated with a change in frequency of stool; 3. Onset associated with a change in form (appearance) of stool. * Criterion fulfilled for the last 3 months with symptom onset at least 6 months prior to diagnosis. “Discomfort” means an uncomfortable sensation not described as pain. Diagnosis of IBS are set only after exclusion of organic pathology. According reсtoromanoscopia and kolonofibroscopia research usually changes absent, just watching some minor congestion, increased vascular injection of the mucosa, the presence of mucus in the lumen of the intestine, which is not a consequence of the inflammatory process, as changes in proctosigmoiditis more pronounced and persistent. According iryhographia observed intermittent, irregular, often painful filling the colon, spastic haustration it, alternating sections of intestine with low and high tone, narrowed and expanded. Emptying the colon insufficient and uneven terrain in the absence of changes in the mucosa. 6 Morphological examination of biopsies of mucosa most informative method for differential diagnosis of IBS and colitis, however, in pediatric practice, this research should be done only in specialized clinics under strict indications. IBS may be accompanied by secondary changes of the mucous membrane of the colon that are always superficial, back in remission and prone to progression as extensions of the disease. Manometry: with balloon stretching of the rectum - Increased pressure. According to research of coprocytogram no signs of inflammation. Fecal fragmented, do not contain food residues doped small amount of mucus. Dysbacteriosis of fecal: І degree (compensated form): a decrease by 1-2 order of bifidobacteria and/or lactobacilli, esherihy; may increase the number esherihy. ІІ degree (subcompensated form): increase number or a representative of pathogenic microorganisms to a level of 105-107 COO/g, or association of pathogenic microorganisms to a level of 104-105 COO/g III degree (decompensated form): a significant lack of bifidobacteria and lactobacilli, numerous associations increase pathogenic microflora in the number 106-107 COO/g or more. Differential diagnosis of IBS and chronic colitis complicated enough. However, typical attacks of abdominal pain, which is usually accompanied by constipation, lack of disturbance of the general condition of the patient, pronounced changes in the intestinal mucosa according rectoromanoskopical research breach tone and motor function of the colon according irrigoscopy can establish the correct diagnosis. Treatment Treatment of patients with IBS include diet, psyhoreflexotherapy, physiotherapy and exercise therapy, phytotherapy. Drug therapy is prescribed only by a long stable course of disease in low efficiency of other methods. Psychotherapy - is difficult to overestimate the importance of the confidential conversation doctor with a child and its parents, which aims to convey in simple terms the possibility of complete cure provided a rigorous implementation of the recommendations the doctor uselyty child and parents faith in recovery. If psycho-emotional changes (feeling of anxiety, sleep disorders, irritability, "recess in the disease," intrusive thoughts) show psychotherapy sessions (rational therapy, autogenic training, a suggestion in the waking state, hypnosis, etc.). The effectiveness of psychotherapeutic effects increases with the combination of psychological and reflexology aimed both at addressing psycho-emotional disorders, and normalization of motor function of the intestine. A necessary condition for successful treatment is the creation of adequate hygiene and dietary regime. Children should be protected from stressful situations, to create a favorable atmosphere in the family and children's team, ensure regular exercise, hydrotherapy, physical therapy sessions. Necessary to control the frequency of bowel movements in a fixed time ("reflex to pot"), which contributes to a rational diet. In the IBS, accompanied by constipation, diet should include the maximum number of vegetable fiber and products that increase peristalsis. Designed diet №3, which includes fruits and vegetables - carrots, , sauerkraut, apricots - mostly raw; milk products - a one-day kefir, yogurt, sour cream, cheese and cream; butter and vegetable oil; rye bread, crisp bread with , meat and fish in any form, including fish and meat soups, preferably in a cool manner; grains - buckwheat, barley, barley; boiled eggs. Recommended honey, fruit, jams, drinks in the cold, in addition to carbon dioxide. Exclude jelly, strong tea, chocolate, pureed oatmeal, muffins, soups mucous. In the IBS, accompanied by diarrhea, a diet excluding foods that promote bowel movements: spices, spicy and savory seasonings, vegetables and fruits, milk, brown bread, fatty meats and fish, pastry, cold drinks. Recommended tea, cocoa, water, white stale bread, crackers, cookies, dried тщт-fancy bread, three-day kefir and yogurt, a little butter, eggs, chicken broth fragile, mucous soups water, rice, oatmeal or blueberry concoctions. In steam meat dishes instead of bread should add rice. Allow chicken and low-fat varieties fish boiled, jelly, jelly with a little sugar (diet №4). Meals give small meals 5-6 times / day. The total duration of this diet 7-8 days, after which the patient is gradually transferred to a broader diet under the supervision of the portability of each product. Phytotherapy in differentiating IBS given the nature of disorders (diarrhea, constipation) and type of motility (hyper, hypomotorical). In the IBS, accompanied by constipation, the basis of treatment is herbs action. By laxatives are substances that cause accelerated promotion and dilution of intestinal contents, causing defecation. On the mechanism of action of laxatives, herbal and natural origin conventionally divided into 4 groups: 1. Medications that cause chemical irritation of the intestinal mucosa receptors and reflex increase peristalsis. These include herbal laxatives containing anthracene glycosides ( root, buckthorn bark, buckthorn fruits, cassia leaves). Under the influence of digestive juices and enzymes of bacteria anthracene glycosides split in the gut, stimulate peristalsis and accelerate the promotion of content. Laxative effect observed after 8-12 hours after application of the drug. 2. Medications help to increase the volume and dilution of intestinal contents and limit the absorption of fluid in it. These are substances of plant origin, are able to absorb water and swell (flax seed, wheat bran, kelp). Substances which swell in water, impede its absorption and lead to moderate laxative effect, by reflex stimulation of intestinal peristalsis increased weight of its contents. 3. Substances which mechanically facilitate the promotion of intestinal content and promote its softening include vegetable oils (olive, corn, sunflower, castor). They have a mild laxative effect, and in the small intestine lipase splits to form , which has a strong irritating effect on receptor and reflex increases intestinal peristalsis.

7 4. Medications that increase the osmotic pressure of intestinal contents (, dufalak, normaze, , glycerin , holitel, , magnesium sulfate). They are not absorbed in the intestines, bind water and increase the amount of fluid in fecal masses, which gives them a soft, mushy texture. In dyskinesia Bowel hyper type widely used herbs with effect: chamomile flowers, mint leaves, fennel fruit, anise and herbs fees. In dyskinesia Bowel hypomotorical type using herbs that contribute to increased intestinal motility: Slany kelp, flax seed, rhubarb root, buckthorn bark, buckthorn fruits, cassia leaves, vegetable oils and phytomixture. Since IBS is often accompanied by signs of dysbiosis, shows the use of (leaves of eucalyptus, sage, calamus rhizomes, fruits, raspberry, mountain ash, blueberry, rose, fennel, caraway, fennel, black currant, herb wormwood) and food (apricots , carnations, garnet, cornelian cherry, cranberry, cinnamon, onions, carrots, radishes, garlic, apples) that causes normalization of intestinal flora. Along with medicinal plants used in pediatric patients as finished dosage forms made from medicinal plants. If constipation is recommended: - juice (1 tsp 2-3 times/day for 20-30 min without food for 15-30 days); - hlaksena (by 1 ½-tab, but not more than 2 tablets per day with meals) - castor oil (5-15 g for an appointment); - kofol (rehulaks by ½-1 cube/day) - buckthorn extract liquid and dry (liquid - 20-40 drops per reception, dry - 1-2 tablets before bedtime) - laminaryd (1-2 tsp granules 1-3 times/day after meal with ¼-½ cup water) - powder licorice root complex (in ¼-½ tsp 1-3 times/day before use mix in water), - pursenid (1-2 tablets before bedtime) - tablets of rhubarb (from 2 years - 0.1 g, 0.15 g 3-4 years, 5-6 years, 0.2, 7-9 years 0,25-0,5 g, 10-14 years 0,5-1,0 g an appointment), dry extract of rhubarb - in the same doses Potions Raven - to ½-1tsp 2 times/day before meals, - seneyd (senade children 1-3 years ½ tab, 3-12 years - 1 tablet before bedtime) - Potions harrow (1 teaspoon 2-3 times/day before meals). For drugs that enhance bowel function, are also prokinetic - cerukal (Reglan), Motilium, perylium, used in treating constipation hypokinetic. In the treatment of hyperkinetic constipation with abdominal pain caused by spasm of the intestine using: • myotropic antispasmodics: drug of choice is mebeverin, duspatalin, which reduces the tone of smooth muscles of the intestine and has direct myotropic antispasmodic effect (from 6 years to 2.5 mg/kg 2 times/day for 30 minutes before meals for 7-10 days); papaverine on 0,005-0,06 g 2 times/day; drotaverine, noshpa 40-200 mg/day in 2-5 receptions; pryfiniya bromide Riabal 1 mg/kg/day for 7-15 days - effectively antispasmodic, acting at all levels of the digestive tract, is released in the solution and tablets; • anticholinergics (Buscopan), antispasmodic effect on smooth muscle is more pronounced. When constipation psychological and neurogenic nature combined with hyper-impaired colon drug of choice is debrydat having enkefalinopodibnyy effect. Available in tablets and suspension. Children over the age of 5 mL per 5 kg/day in 2-3 divided doses. Representative enkephalins are also meteospazmin (consisting of alverynu and simethicone). Available in capsules, older children 1 capsule 2-3 times/day in the presence of hyperkinetic constipation, combined with flatulence. The treatment course of 10-14 days. Many laxatives hypokinetic dyskinesia in preference to those that increase the volume of intestinal contents (laminaryd, normaze, dufalak). Medication for the treatment of chronic constipation is Forlax - osmotic laxative that increases the mass of chyme through the liquid content without changing the biological properties of chyme and faeces preventing loss of electrolytes. Assign within 7-10 days 1 package 1 time in the morning during breakfast with 100-200 ml of fluid. Indications for selective decontamination are signs of intestinal dyspepsia, excessive growth of microorganisms in the small intestine, the presence of inflammation in the intestines and conditionally pathogenic microflora in crops. For selective decontamination using: А/ intestinal antiseptics: • Nifuroxazide: to 6 months - 2.5 ml of 2 time/day, 6 months-6 years - 5 ml 3 time/day, after 6 years - 2 tab 4 time/day; Systemic antibiotics used for selective decontamination in dysbiosis rare. B/ : • Natamycin - 0,05-0,1 g 2 times/day; • Fluconazole - 3 mg/kg/day; and other. C) bacteriophages (the allocation of planting monocultures of microorganisms in high titre): • coli-proteus fah- 30 ml 2-3 times a day inside and 40-60 ml enema; • intectifah- 5-10 ml 3 times daily for 5-6 days; • combined bacteriophage - in doses intestifaha; • polyvalent piobakteriofah - in doses intestifaha; • in the absence of sensitivity Staphylococcal flora bacteriophages - hlorfillipt (1 drop of 1% alcohol. solution per 1 kg per reception 3 times a day, rectally - 1 ml of 1% solution). Restoring normal flora of transmitting agents of prebiotics and probiotics. When disbacteriosis developed clinically manifest with severe immunodeficiency - immune agents (lysozyme, interferon, etc.), administered simultaneously with bacteriophages, probiotics or antibiotics. Enzyme preparations (Creon, panhrol, pancreatin and others) - the dose according to age and body weight. 8 In the IBS, accompanied by diarrhea, widely used binders and enveloping means. If bloating is used antyflatulenty - simethicone preparations. Sedative therapy - if necessary prescribe tranquilizers, or (depending on the syndrome, which predominates in the clinical picture) on the recommendations of neuropsychiatrist. Cleansing enemas are used only for long-term (2-3 days) absence of stool. Uses hypertension (10-20 ml of 10% sodium chloride), chamomile, oil enema, which in turn are used. With frequent urge to defecate recommended enemas of starch. When treating patients with IBS occupy an important place physical factors. When constipation to improve intestinal muscle tone recommend diadynamical currents Amplipuls, galvanization abdominal. Antyspastychnyy influenced by the electrophoresis magnesium sulfate, papaverine hydrochloride, platifillin, dibazolu. If diarrhea is accompanied by spastic symptoms, prescribe warming compress, heating pad on your stomach, or ozokeritovye paraffin baths, inductothermy. Mineral water is recommended to be used after the disappearance of the acute manifestations of the disease. Water should not be combined with herbs. In the IBS, accompanied by constipation, apply highly mineralized sulfate or chloride mineral water. The tendency to diarrhea recommend water with low mineralized. Water used for 30-40 minutes before eating, the tendency to diarrhea - slowly, with constipation - big gulps in a volume of 150 ml, duration of 3-4 weeks. Physiotherapy and massage: used mainly by the presence of constipation. Use exercises that promote rhythmic compression of the abdominal wall muscles (lift straight legs when lying on your back, torso atm etc. ..). Exercise not only stimulates bowel movement, but a positive effect on overall health, his nervous and mental health. Massage stomach (circular clockwise motion duration 8-10 min) especially shows young children. It is advisable to massage preceded defecation. The duration of inpatient treatment of patients with IBS: 7-10 days, may be treated in a day hospital or outpatient. Requiring treatment outcomes: relief of pain, normalization of intestinal motility, recovery qualitative and quantitative composition of intestinal microflora. Medical observation for 3 years extraseizure period. From clinical supervision can be removed in the absence of pathological changes after a full laboratory and instrumental examination. Antirecurrent treatment 2 times a year. - review of pediatric gastroenterologists 2 times a year, a pediatrician - 2 times per year; surgeon fromfor requirement - korpohrama 2 times / year - fecal helminth eggs and protozoa - 2 times per year - dysbacteriosis of fecus - 2 times a year (the first year of surveillance), then the request - - 1 per year (the first year of surveillance, then - on request). Sanatorium treatment of patients with IBS advisable to carry out a local specialized sanatoriums waters of low and medium salinity for drinking water treatment, intestinal lavage, microclysters. Balneology resorts recommended only for long-term sustainable course of the SEC in conjunction with dysbiosis and inflammation.

CHRONIC ENTERITIS Chronic enteritis (CE) - Polyetiological disease which is based on inflammatory and degenerative processes in the wall of the small intestine that causes digestive disorders, suction, secretory and motor functions of the body and, consequently, metabolic disorders. Etiopathogenesis Risk factors for the development of CE in children are: - recurrent disorder treatment and nutrition, toxic factors - infectious diseases (dysentery, salmonellosis, typhoid fever) - transferred before or concomitant chronic diseases of the digestive system - allergy, neuro-psychiatric disorders. All these factors lead to violations of the barrier function of the small intestine, reducing the activity of enzymes membranes, breach the wall cavity and digestive system. In the lumen of the small intestine retained products of hydrolytic cleavage of nutrients (mainly glucose and fructose) that hold water and increase its secretion, as well as a favorable environment for the formation of dysbiosis. The wall of the intestine becomes permeable to proteins, resulting in the development of inflammation violated trophic function of the epithelium of the mucosa, progressive degenerative changes violated digestion and motility, and there are progressive disorders, protein, fat, carbohydrate, vitamin and mineral metabolism, the severity of which correlates with the severity of disease and age children. Classification: pediatric use in working classification of CE І. By origin: ІV. According to severity: - primary - mild - secondary - moderate - severe ІІ. By etiology: V. By course of disease: - postinfectious - monotonous - allergic - recurrent - caused by congenital fermentopathy - continuous-recurrent - postoperative - latent - mixed etiology 9 ІІІ. During the period of the disease: VI. The nature of the morphological changes - exacerbation - surface - subremission - atrophic - remission

Clinic Clinic of CE depends on the severity of the disease. For mild note only local symptoms associated with bowel dysfunction. Moderate flow characterized by the appearance of common symptoms caused by metabolic and functional state of other organs and systems. For heavy flow severity of common symptoms dominates the local. The main clinical manifestations of HE are diarrhea, flatulence, abdominal pain, polyexcrements. Abundant stool, stool frequency from 2-3 to 10-15 times/day. Stool often light yellow color, containing large amounts of mucus and bits of undigested food. Sometimes the stool consistency of clay, gray, shiny, due to the increasing number of fat. At the prevalence in the intestines of putrefactive processes - fetid feces, alkaline consistency; with the predominance of the gut fermentation processes - frothy stool, acid reaction. The abdomen of the patient bloated, increased peristalsis, marked feeling of heaviness and fullness, which decreases after discharge gases. These symptoms are amplified in the afternoon, especially after eating fatty foods, sugar, fruits and vegetables. Sometimes children decreased appetite, even anorexia. When abdominal palpation noted mild diffuse tenderness, most marked on the left and above the navel, rumbling bowel loops. Expressed varying degrees of chronic eating disorders, signs polyhypovitaminosis, fatigue, irritability, weakness, lethargy, headache. Clinical manifestations of the disease are also affected by involvement in the pathological process of other digestive organs (stomach, liver, pancreas, gall bladder). In children there early endogenous protein deficiency, manifested deficiency of body weight, retarded growth, anemia, suppression of the immune system. As a result of calcium deficiency occur increased fragility of bones, osteoporosis, seizures caused by hypocalcaemia. Frequently observed symptoms of food or drug allergy. Most children expressed signs of intestinal dysbiosis due bifidoflory deficiency and increased growth of Escherichia coli with altered enzymatic properties. At the onset of the disease is dominated by symptoms associated with inflammatory bowel changes in the future - signs due to impaired digestion and absorption in the small intestine. Diagnosis When diagnosis is important history. Attention is drawn to the presence of bowel disease in relatives of patients dietary habits, tolerance of certain foods (especially milk, sugar), transferred acute intestinal infections and parasitic diseases. Coprocytogram: creatorrhea (a large number of muscle fibers with preserved structure), amylorrhea (extracellular starch), steatorrhea (increase the amount of fat), resulting from the formation of malabsorption syndrome, chronic enteritis characteristic. Bacteriological took the stool to evaluate the state of intestinal microflora, which is important when selecting appropriate therapy. Determination of enterokinase and alkaline phosphatase in the faeces: increased early in the disease, and the progression of atrophic processes in the mucosa of the intestine - are falling, which is typical of a long and difficult disease. X-ray studies of the digestive tract: the presence of inflammation - deformation folds of mucous membrane that lose normal transverse direction can not be located or traced randomly, unequal width damaged at the edges. With the progression of atrophic changes increased flatness of terrain mucosa, between the folds intervals are not differentiated, the contours are aligned. The typical accelerating the passage of contrast material, indicating that gut dysmotility. Research biopsy of the intestinal mucosa with CE in pediatric practice is rarely used only in specialized institutions under strict indications. Treatment Therapeutic measures for CE include diet, medications to restore motor function and trophic intestine, digestive and transport processes in the intestinal wall, eliminating dysbiosis, disorders of metabolism, enhance immune reactivity. Diet: extra protein, physiological fat, carbohydrate restriction. During exacerbation in order to preserve the mechanical bowel prescribed hungry pause for 8-12 hours followed by fractional meals 5-6 times/day. From diet excluding foods that adversely affect the condition of the intestine, especially milk, gluten, disaccharides, raw fruits and vegetables, brown bread. For food dosed infants use dairy products. Older children consistently prescribe treatment tables №4 (3-5 days), №4b (10-12 days), followed by the transition to table №4c. Increased amounts of protein is achieved by appointment cooked meat and fish, mashed cottage cheese, protein omelet. After removal of acute inflammatory changes appropriate destination enpitu protein that increases the protein content in the diet by 10%. The drug is used as a 15% solution (for children up to 3 years - 100 ml, 3-7 years - 150 ml, over 7 years - 200 ml 1 time/day for first dinner or 2nd breakfast). Begin entering enpitu protein with ¼-½ the maximum level and within 3-4 days the dose was increased to the required, which is used for 4-6 weeks. The amount of fat limit only heavy flow CE. Useful food enrichment in polyunsaturated fatty acids (vegetable oils), which are good for portability you can substitute butter ⅓. Portability depends on the amount of carbohydrates and fat cell membranes, and also features hemicellulose. Recommended vegetables, fruits, whole grains, which contain little fiber and cell membranes. It is necessary to use special methods of cooking (boiling water, steamed, mechanical grinding products). For diet №4 recommend following products and foods: dried white bread, pureed soups precarious beef broth, chopped cutlets of meat and fish, rice or semolina cooked in water or milk, eggs (cooked boiled first, further - cool), sugar

10 (40 grams per day), jelly, jelly with fruits and berries are high in tannins, decoctions of blueberry, rose, black currant juice, lemon, butter (added to the dish 5 g). Diet №4b physiologically full content of salt 8-10h. Administered in the diet yesterday's bread baking, biscuits or scones nezdobni 1-2 times/day, soups precarious broth (meat or fish) with well-boiled cereals or pasta and chopped vegetables (potatoes, zucchini, carrots, cauliflower pumpkin); tender meat and low-fat varieties of fish, chopped or a piece of cooked steamed or boiled; dishes and side dishes of these vegetables boiled or shabby form and in the form of steam souffle; various cereals (except millet and barley) in water with ⅓ volume of milk, steamed puddings with eggs; jelly and cream compotes, jellies, soufflé, mousse with sweet fruits and berries; for good portability allows a small amount of sweet berries raw (strawberries, strawberry, raspberry), juices, diluted with hot water; dairy products with low acidity, mild cheese, cheese in kind, grated, also in the form of steam or baked puddings; sauce, milk and fruit; add to butter dishes or make bread in small amounts (5-15 g). In remission patients transferred to №4с table, which is designed to provide adequate nutrition, maintaining state compensation for bowel disease and related diseases of the digestive tract. Allow dishes baked in the oven, mainly in meat to form not broken. For good tolerance to soups or garnishes add , green , young beans, beets, ripe tomatoes raw (100-200 g / day), lettuce with sour cream, crisp cereals (except millet and barley), finely chopped pasta, ripe tangerines and oranges, watermelons, sweet, fruit and berry juices sweet varieties (, cherry, strawberry), with good tolerability - milk in its natural form. For stable compensation process and the absence of concomitant diseases of the digestive tract appointed common rooms. To eliminate polyhypovitaminosis with CE prescribe vitamins in doses that far exceed the need, preferably parenterally. Especially show B vitamins, which favorably affect the secretion and intestinal motility, normalize its enzyme activity, with antitoxic action. Assign thiamine - 10 mg riboflavin - 10 mg pyridoxine - 10 mg, folic acid - 1 mg, nicotinic acid - 5.10 mg cyanocobalamin - 50-200 mg per treatment 15-20 injections. Daily dose of 200-300 mg of ascorbic acid, retinol 2 mg. Adsorbents, binders and enveloping means: reacting with tissue protein surface inflammatory changed mucosa they form insoluble film covering a thin layer of mucous membrane of the digestive tract and protects it from mechanical, thermal and chemical stimuli influence enzymes. This reduces the permeability of the walls of blood vessels, swelling, redness, subsiding inflammatory process. Binding of plant origin have expressed mild bacteriostatic and bactericidal effect. To enhance the efficiency of astringents administered 20-30 minutes before meals. By herbal astringents include oak bark herb St. John's wort, Highlander snake rhizome, alder cones, sage leaves, chamomile flowers, fruits, cherry, rhizome erect cinquefoil, grass cattle. By enveloping include starch, flax seed, marshmallow root, rhizome with the roots of licorice. Anti-inflammatory effect with herb St. John's wort, chamomile flowers, leaves of sage, calendula flowers. Ready medications: bismuth preparations, Imodium, tanalbin, romazulan, Enterol age in doses. - romazulan has anti-inflammatory and antispasmodic action of light, reduces bloating. The drug is taken orally ½ teaspoon diluted in a glass of hot water. For enemas diluted 1.5 tbsp preparation of 1 liter of boiled water. - tanalbin has astringent and anti-inflammatory effect. Apply 0.1-0.5 g depending on the age of the child 3-4 times/day. - Imodium has a strong effect, it selectively affects the muscles of the small intestine, thereby increasing the density of feces, decreases the number of bowel movements. Appointed 1 capsule 10-20 drops or 5.10 ml of syrup 2-3 times/day. - enterobene has a similar effect. Tablets 0,002 g, appointed children of 8 years. Early treatment of acute diarrhea and after emptying prescribed 1 tablet. Daily dose of 4 tablets (8 mg). Chronic diarrhea dose is 1 tablet. Apply without chewing with a little water. - Enterol - antidiarrhoeal agent of biological origin that has an antagonistic effect on pathogenic and conditionally pathogenic microorganisms that cause diarrhea. Assign 1-2 capsules or sachets a day. With the increased gas and bloating - antyflatulenty based simethicone. Simethicone dose depending on the age of 15-25 drops 3-5 times / day before or with meals. Enzyme preparations containing pancreatic enzymes and bile acids (pancreatin, panzinorm, Festal, Creon), to appoint ½-1 tablet 3 times / day with meals, treatment for 2-3 weeks. Indication for antimicrobials in CE is the lack of effects with diet, binders and enzymes. Widely used eubiotics who have pronounced antibacterial effect on conditionally pathogenic microflora (enteroseptol, intestopan, meksaze, meksaform). If the body foci of chronic infection used nitrofuran drugs (furadonin, furazolidone), drugs nalidixic acid (blacks, nevigramon), azo compounds of salicylic acid with sulfapyridine (sulfasalazine, sulfapirydazyn, salofalk), antibiotics (polymyxin M, ampicillin). In acute disease with marked diarrhea and there is evidence of toxicity exycosis spend infusion therapy. To eliminate heartburn, itching in the anus area, resulting from prolonged diarrhea, rectal prescribed procedure - microclysters candles. Clinical supervision After discharge from the hospital during the period of convalescence the patient continues to use enzymes, vitamins, probiotics. Overview doctor for 6 months - per month with coprogram, then 1 time/quarter. In remission (no earlier than 1 year after exacerbation) 2 times/year by antirecurrent treatment for 1 month (fees herbs, vitamins - thiamin, pyridoxine orally or parenterally, enzymes, probiotics, Methyluracilum - 10 days). Medical examination and coprogram - 2 times/year. During stable remission review 1 time/year. The child is removed from the dispensary, not earlier than 3 years after achieving stable remission. 11

CHRONIC COLITIS Chronic colitis (ChC) (chronic ulcer colitis) – code K59.9 – a chronic inflammatory-dystrophic lesions of the mucous membrane of the colon dominated the clinical picture of signs of functional disorders. The frequency of HC in children is 5-12 1000 children. ChC problem, despite numerous studies, it is difficult. For a long time this term signified a variety of pathological conditions of the intestine. According to modern concepts ChC – a morphological concept, characterized by a combination of characteristic bowel disease pain and dyspeptic syndromes with histological confirmed signs of degeneration of the epithelium, a decrease in deep crypts and infiltration limfoplazmocytic varying severity. Etiology and pathogenesis Development ChC numerous factors contribute to the external and internal environment: – Psycho-emotional stress that causes functional disorders of the bowel (irritable bowel syndrome), and later - and the emergence of ChC; – Violation of the diet; – Infectious disease (of particular importance is the aggressiveness of the pathogen, late and incorrect treatment); – Reducing the reactivity of the children – Increased allergy of the body, which can be both a cause of illness and accompany her due to lack of local remedies; – Gastroenterological diseases that carry a reflex effect from the center of the primary lesion to the damage neurohumoral regulatory mechanisms; – Congenital diseases and abnormalities of the intestine; – Transferred somatic and surgical diseases; as well as their use for the treatment of antibiotics, sometimes unnecessarily high doses and, consequently, violations of ecological community intestine. Due to changes in the microflora is intense multiplication of opportunistic microorganisms that actively use nutritional components. Bacterial amino acids – indole and skatole metabolites – have a pathogenic role in causing inflammation in the intestinal mucosa. Because life opportunistic microorganisms increases the amount of histamine that causes sensitization, inhibition of cellular and humoral immunity. The ability to adapt to environmental conditions, conditional pathogens pose competition usual intestinal microflora. Deficiency of bifidus flora causing disturbance of digestion, absorption, assimilation of nutrients. Equally important in the pathogenesis of ChC with disorders of the nervous regulation of intestinal. The basis of these disorders is weakening the inhibitory effects of the cerebral cortex in CNS sections below are for a significant reduction in the reactivity of the sympathetic autonomic nervous system level, and an increase in blood biologically active substances - serotonin and histamine. Play an important role as a compromised immune reactivity. When the content of immunoglobulins in the blood of patients exhibit autoantibody to tissue of the intestine. This indicates a role in the pathogenesis of the disease sensitization to its own tissue antigens. Significant changes occur in the circulatory system: Disturbed permeability of blood vessel walls – There are signs of DIC – activation of coagulation system of blood formation microtrombs which leads to poor circulation, formation of erosions of the mucous membrane of the intestine. Risk factors for transformation of irritable bowel syndrome in HK are: – Duration of disease more than 5 years; – Violation of biocenosis colon intensive multiplication of opportunistic microorganisms; – Reduction of IgA; – Increasing the number of undifferentiated lymphocytes; – A modification to the activity of lipid peroxidation and antioxidant protection. Classification Multiplicity of pathogenetic mechanisms of disease causes difficulties ChC classification and originality of clinical signs of disease. I. Etiology: IV. The severity of the course: - nutritional - mild - infectious or parasitical - moderate - when exogenous and endogenous intoxications - severe - with endocrine disorders - in diseases of the central nervous system II. Morphology: V. Period of the disease: A/ endoscopy: - aggravation - catarrhal - partial clinical remission - catarrhal, follicular - complete clinical remission - erosion - clinical and endoscopic (histological) remission B/ histology: - without atrophy - with atrophy (initial, moderate, severe)

– inactive – active (mild, moderate or severe activity) III. The localization process: VI. The character disorder of motor function of the colon: - proctitis - hyperkinesias-hypertension 12 - right sided colitis - hyperkinesias-hypotonia - left-sided colitis - hypokinesia-hypertension - total colitis - hypokinesia-hypotonia - transverzitis Clinic ChC course in children is characterized by vague clinical symptoms, especially in the early stages of the disease, there is a long, prone to recurrence of the course, which leads to some difficulties early diagnosis. For sharpening ChC characteristic changes being patient, the appearance or increase of symptoms of chronic intoxication: increased fatigue, loss of appetite, headache, subfebrilitet. At the same time, mark infringement stool. At long delay emptying there is pain in the left iliac region associated with the overflow of the colon, which disappears after a bowel movement. Stool in small amounts, often in the form of "sheep" of feces, sometimes at the end of defecation impurities appear bright red blood, which is caused by the presence of cracks anus. For prolonged constipation nausea and vomiting caused by fecal intoxication. ChC may be associated with increased bowel movements to 3-5 times/day, a change in stool consistency, admixtures of mucus, flatulence, pain in the lower abdomen along the colon. Review During the inspection show signs of mild chronic intoxication, imposing white tongue stratification. Abdomen swollen, painful on palpation in the region of the sigmoid and descending colon, which was caused mainly left-sided localization of the inflammatory process. Typical positive symptoms exemplary, Hertz, "column of air". Peculiarities of ChC in children determined by the nature of disturbances in motor function of the colon. Depending on the type of dyskinesia stool and abdominal pain are some of the features. When combined with colitis enteritis emptying liquid, abundant, much pronounced flatulence are often described exemplary symptom, pain at the point Porhesa. Course ChC long, undulating, with alternating periods of exacerbation and remission. Diagnosis To verify the diagnosis and choosing the right treatment strategy is necessary, first, to differentiate inflammatory and degenerative and functional disorders of the intestine. Most informative method of diagnosis of HC is a sigmoidoscopy (colonoscopy) with biopsy and aspiration biopsies morphological study. Complete blood count: possible manifestations of moderate anemia, eosinophilia. Endoscopy: intestinal mucosa edematous, hyperemic; vascular pattern unclear or rebuilt, which corresponds to catarrhal signs proctosigmoiditis. The presence of numerous hyperplastic lymphoid follicles indicates catarrhal, follicular proctosigmoiditis. Morphologically marked: – proliferative changes – lowering the surface and glandular epithelium, the presence of dense limphohistiocytic infiltrate with many lymphocytes and eosinophilic granulocytes; – signs of edema – changes in the epithelium of crypts, decreased vascular tone, increase blood supply. In modern conditions the diagnosis of HC should be based on histological examination of data, since the results of endoscopic and radiological examination is not always possible to verify the presence of inflammation. Morphological examination of the intestine should be carried out under strict indications in a specialized department. Conducting irrygographic helps identify motility disorders and birth defects and developmental abnormalities of the intestine, against which there is often a HC. Indirect radiological signs of inflammation of the intestinal mucosa include: restructuring the relief of mucous membrane folds discontinuity, the presence of flocculation, indicating an excessive amount of mucus in the lumen of the intestine, alternating areas of spasm and hypotension, rigid walls. HC usually is accompanied by intestinal biocenosis, it necessitates examination of stool for detection of dysbiosis. Secondary importance in the diagnosis of HC has expanded coprology study that allows us to estimate the processes of digestion and absorption in the intestines, and according to the reaction Triboulet-Vyshniakova - an inflammatory process:  ileocecal syndrome: - with the benefits of fermentation: foam stool golden color, pH is acidic, intracellular starch jodophilic flora - with the benefits of rot: brown, putrid odor, pH - alkaline, no jodophilic flora  colitic syndrome unformed stool, mucus, increased white blood cells, epithelial cells of varying degrees of degeneration. ChC differentiate from chronic enteritis and irritable bowel syndrome. Treatment Treatment of ChC should be differentiated, comprehensive and include diet therapy, drug therapy, local rectal procedures, and physio-balneotherapy. 1. Diet. To reduce intestinal motility and agitating influence of food on its mucosa prescribe fractional food 6-8 times / day. At the prevalence of diarrhea - temporarily excluded or limited products that increase intestinal secretion and motility (№4 diet and its variants - №4b, №4c). To eliminate bloating limit the amount of carbohydrates, milk and dairy products. At the prevalence of constipation – a diet rich in fiber (beet, carrot, pumpkin, prunes, apricots, fruit and vegetable juices). Effective destination bran In remission designate common table with the exception of fatty meat, spicy and savory dishes, extract, fancy pastry. 2. Medical Therapy 13 Prescribed drug therapy given the nature of inflammatory changes, type dysbiosis, a variant of motor-evacuation disorders.  antimicrobials drugs appointed with long, that does not decrease symptomatic agents of diarrhea in exacerbations of chronic foci of infection, inflammation, combined with pronounced symptoms of intestinal dysbiosis. - 5-ASA drugs: salazopirydazyn, mesalazane (section ulcerative colitis) course 5-7 days in middle-dose - Nitrofuran drugs (furazolidone 10 mg/kg/day, Nifuroxazide).  symptomatic therapy - depending on the nature dyskinetychnyh bowel disorders and character of stool: a/ with constipation, caused hyperactivity disorder: - Sedatives (drugs valerian, bromine, etc. in age dose rate of 1-2 months) - Spazmolitics (section irritable bowel syndrome) b/ with constipation, caused hypokinetic disorders: - Drugs that regulate intestinal motility (section irritable bowel syndrome) - Laxatives (preferred drugs with hydrophilic effect – lactulose, see section laminarid IBS) c/ with the express syndrome of diarrhea: - Adsorbents, anticholinergics, enzymes (section irritable bowel syndrome)  correction of intestinal dysbiosis - see section irritable bowel syndrome.  desensitization therapy - mindful of the role of allergic factors in the pathogenesis of ChC  vitamin: inflammatory changes in the mucous membrane of the colon, as well as related inhibition processes of digestion and absorption contribute to the emergence polihypovitaminosis, and therefore shows the inclusion complex of therapeutic measures (ascorbic B vitamins, folic and nicotinic acid)  stimulators of reparative regeneration, characterized by non-specific anti-inflammatory effects (for example methyluracilum) – according to experimental and clinical studies, marked by their high efficiency in the complex of therapeutic measures in chronic inflammatory bowel disease. A course of treatment for 6-8 months promotes elimination or significant reduction of clinical signs of disease, severity of inflammation in the lining of the intestine, long-term (1-3 years) remission. Methyluracilum appoint 0.25-0.5 g 3 times/day during or after a meal.  local therapy for ChC includes the use of enemas and suppositories, which have anti-inflammatory effect on the mucous membrane of the colon. Use microclysters of chamomile, Collargolum, with atrophic changes and subatrofichnyh - oil (fish oil, vegetable oil). The use of candles reduces intestinal spasm, pain in anus, emptying. Candles administered at night or in the morning before bowel movements. Use the finished product antyhemorrhoidal candles - anuzol, neoanuzol, betiol etc.  physiotherapy, which have anti-inflammatory and helps eliminate intestinal motility disorders: - heat treatment: application to the abdomen paraffin wax (to improve blood supply to the intestine, reduces the severity of inflammation); - diathermy (with a special rectal tip): helps normalize intestinal peristalsis, eliminates pain; - depending on the type of disturbances in motor function of the intestine prescribed electrophoresis of mud solutions and drugs: a tendency to constipation dominated spastic component used anticholinergics, hypotension intestine - a calcium supplement.  herbal medicine for chronic bowel disease is carried differentiated according to the nature of the violation of its activity (constipation, diarrhea) and the type of motility (hyper, hypomotoric). Phytotherapy colitis advisable to appoint in the waning inflammation using collections of herbs. 1.Kory buckthorn 10g, 20g elder flowers, fennel fruit 10g, 10g anise fruits. One tablespoon collection pour a glass of boiling water, heated for 15 min in a water bath; infusion cooled for 45 min at room temperature and filtered. Apply ½ cup morning and evening on an empty stomach at bedtime. 2.Plodiv buckthorn 10g, 10g licorice roots, fruit tmynu10h. Preparation and application of the same. 3.Lystkiv 20g, 20g dandelion root, valerian rhizome 10g, 10g nettle leaves. Preparation and application of the same.. These fees are used in conjunction with colitis constipation.  balneotherapy at HC is feasible, widely proposed for use in pediatric patients. Designed balneotherapy in remission or not earlier than 2-3 months after acute disease. Drinking mineral water helps to normalize motor-evacuation function of the intestines, improves digestion and absorption processes, improves the function of the secretory glands of the intestinal mucosa. Use mineral water of low or moderate salinity, rich in sodium sulphate and magnesium salts that affect the nervous and muscular system of the intestine. In HC, accompanied by diarrhea, mineral water prescribe less, 1-2 times / day, always hot. The use of warm mineral water is shown in HC, accompanied by spastic constipation. If you violate motor function of intestinal mineral water should be drunk cold, as it contributes to the stimulation of intestinal peristalsis. Apart mineral water inside, diseases of the intestine are widely used for washing the intestines, intestinal soul. Washing helps to remove intestinal feces, mucus, toxins. Mineral water, acting on the mucous membrane and receptors in the distal intestine, reduces the severity of inflammation, normalize intestinal microflora and motor-evacuation function. Contraindications for the rectal procedures have increased the vulnerability of rectal mucosa, the presence of erosions, fissures of the anus.  physiotherapy – in a subsiding inflammation. In HC prevalence of constipation and spasm pay special attention to the choice of starting position (knee-elbow position, lying on his back with legs bent at the knee joints of the lower limbs). When lowering the tone of the intestinal prescribe exercises for the abdominal muscles in different initial positions. Duration of hospital treatment – 10-12 days. Clinical supervision 14 Clinical examination of children with HC involves a systematic examination of active, antirecurrent treatment, creating conditions conducive to the full recovery. After discharge from the hospital should continue the use of vitamins (B, ascorbic, folic) for 3-4 weeks. When combined with HC enteritis prescribed enzymes (pancreatin, panzinorm, Creon) for 1-2 months, sustainable dysbiosis – probiotics for 1.5-2 months. Phytotherapy for 2 weeks every month for 6 months (changing collection of medicinal plants). Mineral water due to bowel function within 1 month. Review: - pediatrician 2 times/year, children gastroenterologist (first year of supervision 2 times/year, then – 1 time/year) - LOR, dentist, surgeon and other specialists – on request - antirecurrent treatment 2 times/year in a hospital or outpatient. Assign vitamins (B, ascorbic, nicotinic acid), eubiotics, probiotics, herbal medicine, after - mineral water. - CBC and urinalysis 2 times/year - coprocytogram – 2 times/year - fecal helminthes eggs and protozoa – 2 times/year - biochemical feces (reaction Gregersen, Triboulet) 1 time/year - proteinogram, acute phase indicators – 1 time/year - fecal bacteria overgrowth – 2 times/year in the first year of surveillance, more on request. - sigmoidoscopy – 1 times/year (if indicated) - ultrasound of abdominal cavity organs – if indicated. Medical observation for 3 years without acute period of disease. From clinical supervision can be removed in the absence of pathological changes after a full laboratory and instrumental examination. Spa treatment appropriate to carry out local spas and spa resorts of Ukraine (Berezovsky mineral water Morshin, Myrgorod, Transcarpathian group resorts, Truskavec, etc.) in remission in the absence of severe bowel dysfunction: water low and medium salinity for drinking water treatment , intestinal lavage, microclysters.

ULCERATIVE COLITIS Ulcerative colitis (UC) - 51 K cipher - a chronic disease with progressive relapsing course, with periods of bloody diarrhea, which is based on inflammation of the colon with marked destructive changes of the mucosa and submucosal layer. Etiology UC – Polyetiological disease. Risk factors for its occurrence is the genetic characteristics of the child, genetic predisposition (find more antigens HLA DR2, B5, and set specific forms antineutrofilic cytoplasmic antibody pANCA), sensitization of various antigens trauma. The frequency of UC 20:100 000 population. Pathogenesis The most important part of the VC is changing with the formation of intestinal microflora dysbiosis, autoimmune processes, delayed type hypersensitivity reaction, microcirculation disturbances in the intestinal mucosa, psycho-emotional disorders. As a result of inhibition of the protective function of the phagocytic system in the intestine significantly increases the number of pathogenic and opportunistic microorganisms, contributing to a massive flow of blood from microbial metabolites of allergic and severe toxic reactions. During contact with a specific antigen (bacteria, parasites, food ingredients, autoantigens different types) is a formation of sensitized T lymphocytes, which leads to the emergence of delayed type hypersensitivity reactions. Antigen mucosa of the large intestine in the body produce antibodies that cause inflammation and processes close to a crisis of rejection. Marked increase in the concentration of IgG and IgA in lowering serum. Changing the state of cellular immunity: reduced absolute number of lymphocytes, disrupted their ability to blasttransformatsiyi, reduced content of T-suppressor lymphocytes, and T and B lymphocytes in response rosette inhibited phagocytic function of neutrophils. Due to damage to the wall of the colon occurs chronic inflammation with damage to the mucosa, submucosal layer, muscle layer and lymphoid tissue, and inflammation becomes necrotic, erosive, often complicated by bleeding, and perforation with subsequent peritonitis. Typically, lesions show all colon – rectum of ileocecum valve to the cecum. In the event of relapse AML important role played by stress. Pathomorphology The pathological process in UC starts usually in the rectum and extends in the proximal direction. Inflammation may be limited to the rectum (proctitis), straight and sigmoid (proctosigmoiditis) to capture the downward (left-sided colitis) or the entire colon (total colitis). Defeat is not focal and diffuse in nature, it is manifested by inflammation: swelling and plethora, thickening and smoothness folds. In the initial stage of obvious defects can not be, but as the disease progresses there are superficial erosions and ulcers of different sizes, irregular shapes. Prolonged course against the backdrop of ulcers detected psevdopolipy – Land regeneratory hyperplasia. Macroscopically at UC: infiltration of the mucosa and submucosa, vasodilation, inflammation of crypts forming crypt-abscesses, areas of necrosis of the epithelium. Long flow VK accompanied by substitution of normal mucosa scar tissue, narrowing and shortening of the colon, loss haustration, eventually colon takes the form of a hose (symptom "of rubber" or "water pipe"). Classification UC (Lukyanova AN, MF Denisova, 2004) 15 I. Clinical forms: VI. Complications of the disease: 1 Mild 1 Local (intestinal): 2 Moderate - Intestinal bleeding 3 Severe - Bowel perforation with peritonitis occurrence - Toxic dilatation of the colon II. The condition: - Stricture of the colon 1 Lightning - Perianal fistulas 2 Acute - Anorectal complications (anal fissures, anal 3 Chronic: sphincter weakness, , warts, a) recurrent paraproctitis) b) continuous-recurrent - Intestinal dysbiosis III. Stage of disease: - pseudopoliposis 1 The active phase - the period marked clinical - Colon cancer manifestations 2 System (extraintestinal): 2 The period of regression of disease - Liver damage (hepatitis) 3 Clinical remission - Primary sclerosing cholangitis 4 Complete clinical and laboratory remission - Eye disease (uveitis, iridocyclitis, etc.). IV. Prevalence of pathological process: - Aphthous stomatitis 1 Distal colitis - Nodulated erythema 2 Segmental colitis - Pyoderma gangrenosum 3 Total colitis - Sepsis V. The degree of inflammatory activity (determined on the - Arthritis, sacroiliitis; basis of clinical, endoscopic and histological features): 1 first degree 2 2nd degree 3 3rd degree Clinic UC equally often in any age, boys are sick more often than girls. Disease onset in most patients gradually. Only in rare cases, observed acute course in which the clinical picture unfolds entirely within 1.5-2 weeks. As a rare exception observed fulminant disease (clinical picture unfolds quite a few days of onset) is characterized by exceptional severity, gives complications. Usually, the first symptom is the appearance of blood in the stool decorated or mushy. Less often first appears mushy or liquid stools without pathological impurities and only ≈1-3 months - impurities of blood and mucus in the stools. Dysentery-like beginning with rapid progression of toxicity and diarrhea with mucous and bloody feces are rare. In any case, blood and mucus in stools – the main symptom UC. Getting the disease is accompanied by normal body temperature (sometimes – subfebrile). Disturbing weakness, loss of appetite, weight loss. Stool frequency varies depending on the severity of the disease. Concerned about tenesmus, abdominal pain associated with defecation. Sometimes in early disease marked arthralgia, skin rash type anulyarny nodulated or erythema. Patients with UC have some features of psycho-emotional sphere: stubbornness, emotional lability, isolation, sometimes aggressive, some children may be psychopathy, hysteria. Complete blood count: anemia, increase of ESR. Biochemical parameters: dysproteinemia with decreased albumin, dysglobulinemia with increasing α1, α2 and γ- globulins. Increased levels of CRP and sialic acids. Characteristics severity UC depending on the severity of clinical manifestations: Colitis mild: - Overall condition is violated - Normal body temperature - No underweight or moderate weight loss on a background of moderate loss of appetite - After defecation and before eating in the left abdomen occurs paroxysmal pain of moderate intensity, caused by irritable bowel spasm - Diarrhea least 4 times / day - The presence of a small amount of blood and mucus in the stool as separate clots or strokes due to increased bleeding irritable colon mucosa - Objective: pale skin, sensitivity to palpation of the intestine, especially the sigmoid colon - Anemia and degree, accelerated erythrocyte sedimentation rate of 15-30 mm/h - Endoscopy – distal colitis with a small degree of activity. Colitis moderate severity: - Overall condition is broken: weakness, irritability, fatigue - Unstable subfebrilitet - Loss of appetite, nausea, weight loss (underweight 10-15%) and long-term course of the disease - lag in physical development - Abdominal pain during bowel movements or to: the left iliac area, mesogastrium, hypogastrium - Tenesmus, mendacious urgency, flatulence

16 - Diarrhea 5-6 times / day with macroscopically visible blood and mucus - Objective: marked pallor of the skin, decreased turgor tissue sections of colon palpation painful, spasmodic, sealed - Anemia second degree (related not only to blood loss, but also autoimunogemolisis), accelerated erythrocyte sedimentation rate of 25-50 mm / h - Endoscopy – left-sided colitis of moderate activity. Colitis severe: - Overall condition is broken (heavy): severe weakness, indisposition, laxity - In the majority of patients with fever (can be up to 39 ° C) - Loss of appetite even anorexia, marked weight loss (underweight than 15%), the long course of the disease - lag in physical development, slow growth - Intense pain, paroxysmal around the stomach regardless of the act of defecation and eating - Tenesmus, mendacious urgency, flatulence - Diarrhea 8-10 or more times/day, if tenesmus sometimes impossible to determine the frequency of bowel movements. Significant amount of blood, feces often looks like a bloody mass smelly with mucus and pus. - Objective: pale and dry skin, decreased tissue turgor, labile pulse, tachycardia, muffled heart tones, blood pressure reduction. Palpation of the abdomen: local muscle tension in painful zone (defense muscular), marked tenderness segments of the colon, rumbling, clapping noise. - Anemia II-III degree, leukocytosis, accelerated erythrocyte sedimentation rate of 50-70 mm / h - Hypoproteinemia, dysproteinemia. - Endoscopy – total colitis with a maximum degree of activity. - Severe colitis often accompanied by complications of local and systemic.

Diagnosis UC diagnostics is based on a careful analysis of the clinical history (detection of genetic predisposition, intolerance to certain foods, allergies). Assessing the patient's complaints, special attention should be paid to the frequency and nature of stool. In addition to the general inspection, palpation of the colon, it is necessary to examine the anal area, to digital research rectum. Paraclinical criteria: Required laboratory: - CBC (anemia, accelerated ESR, leukocytosis may be) - Urinalysis (no change) - Сoprocytogram (positive occult blood reaction – Gregersen’s reaction, the presence of leukocytes, mucus) - Proteinogram (dysproteinemia with hypoalbuminemia, increased α1, α2 and γ-globulin) - The contents of Ig in serum. Ancillary laboratory: electrolytes, transaminases, total bilirubin and its fractions. Required instrumental methods:  ultrasound of abdominal cavity organs: increased liver size, its seal.  endoscopy: sigmoidscopy or kolonofibroskopy with histological examination of biopsies of the colon. Endoscopic criteria of UC activity The degree of activity Characteristic mucosal And the level of activity Erythema, moderate edema, slight granulation mucosa, attenuation (loss) vascular pattern, (mild) mild contact bleeding II degree of activity Isolated ulcers, erosions, pseudopoliposis, mucosal edema, granularity, lack of vascular (moderate) pattern, significant contact bleeding III degree of activity Significant swelling, lack of vascular pattern, diffuse contact bleeding, grain, solid (severe) purulent plaque ulceration, erosion, pseudopoliposis, free pus and blood in the lumen of the intestine. In remission: reduced severity of inflammatory changes, stored grain and light the vulnerability of the mucosa, blurred vascular pattern, areas of mucosal atrophy, may form scars and pseгdopoliposis. Colonoscopy study more informative because it allows you to inspect all parts of the colon until ileocecum valve. Histologically: degenerative and atrophic changes of the surface epithelium with areas of ulcers, erosions and microerosionі, changing forms of crypts, decreased mitotic activity of epithelial cells, fewer or no bokalopodibnyh cells that produce mucus, neutrophilic and eosinophilic infiltration, reducing the number between epithelial lymphocytes (mainly T lymphocytes).

Severe form of UC rectum Severe form of UC sigmoid colon Inflammatory polyps (psevdopolipy) 17 of the colon caused by of UC

 X-rays can not only establish the presence of inflammation, but also to determine its prevalence and nature of motor disorders of bowel function (irrigography with contrast barium sulfate suspension or double contrast barium sulfate suspension and air): - Local hypermotilyty (accelerated release of the affected area of contrast material, often painful sensations) - Violation haustralyty pattern (asymmetry, deformation to extinction), shortening of the intestine and the offset natural curves, contours serration. In conducting the dual contrast detect discontinuity contours intestine (bowel syndrome bypass ratio). - Changes in the mucosa of relief: the presence of coarse thickened longitudinal folds ("combed" relief), spotted ("marble") photography in places erosions and ulcers – concentrations of barium suspension. The method is used mainly in remission. Consultations related professionals: optometrist, surgeon, oncologist (with prolonged duration, presence of dysplasia in mucosal biopsies of the colon). Differential diagnosis Differential diagnosis of of UC spend with dysentery and other types of bacterial colitis, granulomatous colitis and chronic ulcer, diseases anal area. Acute bacterial dysentery and other acute bacterial colitis, particularly caused by pathogens of Campilobacter, characterized usually by acute onset of severe signs of intoxication and hemocolitis Such a beginning in of UC observed rarely; in most patients the initial period characterized by the appearance of UC impurities (blood, phlegm, pus) issued to the stool in the absence of signs of intoxication. In patients with of UC antibiotic therapy is usually ineffective; According sigmoidoscopy, unlike acute colitis show increased bleeding and granularity of the intestinal mucosa. When you rectoromanoscopy study after 10-14 days in the presence of UC is not observed positive dynamics, whereas in acute colitis in the background of positive dynamics of clinical symptoms noted a decrease or disappearance of the severity of congestion and edema of the mucosa. Granulomatous colitis or Crohn's disease, are often very similar to the of UC clinical symptoms. Both diseases have immunopathological etiology without unexplained etiology. Unlike of UC, Crohn's disease can involve different parts of the digestive tract: more often – cecum and distal ileum, but may be lesions of the left colon, along the small intestine as well as in the esophagus, stomach and duodenum 12. For Crohn's disease is characterized by a clear zone of delimitation destruction from healthy tissue, alternating pathological and normal areas ("jumping kangaroos"). In Crohn's disease inflammation captures the entire thickness of the bowel until the serous layer, accompanied by local lymphostasis, arteriolar occlusion. The process extends from the inside of the bowel wall thickness as ulcers in Crohn's disease is always deep, aphthous or slit-shaped (fissure), characterized by swelling and thickening of the wall, loss of vascular pattern, as a result of all this relief takes the form of "Bridge" developing external adhesions and fistulas. Histologically for Crohn's disease is characterized by transmural lesions of the bowel wall and the formation of nonspecific granulomas containing giant cells Pirogov-Langhans. The clinical picture of Crohn's disease can be a lot to do with VC (diarrhea in 80% ground - 50% loss of body weight - 85% retention rate - 35%), but the disease can be more flabby and torpid, long blood loss may be hidden. Due to the more frequent localization process in the ileocecal region, can be a pain in the right iliac region, which is usually perceived as appendicitis. Pretty typical lesions of the anal and perianal areas (chronic deep fissures, fistulas, paraproctitis). The disease is characterized by a slow but relentless progressive course, responds poorly to therapy. Crohn's disease is characterized radiologically segmental lesion narrowing ulcers, it often formed bowel stenosis. Chronic ulcer colitis (proctosigmoiditis) in children sometimes accompanied of hemocolitis. Unlike UC, for this form of colitis is characterized by severe intoxication significantly accelerated ESR, frequent relapses, and with adequate treatment and noted rapid improvements of patients. According proctosigmoidoscopy not observed contact bleeding is more common subatrophic changes mucosa; erosions and ulcers formed the rarer. Differential diagnosis of UC disease anal area (colon polyps, hemorrhoids, rectal hemangiome, papillom) does not cause much difficulty, provided a qualified review of this area and of rectoscopy. A characteristic clinical feature of these diseases is bleeding, which appears suddenly, in a sufficiently large quantity of bright red color. The nature of the stool is not changed, mucus in stools available. Treatment The aim of treatment: remission, prevention of exacerbations and complications. In acute prescribe bed rest, provide mental calm the patient. Clinical nutrition should provide physiological need for nutrients, help reduce the severity of inflammation, food sensitization, normalization of the functional state of the intestine and stimulate regeneration. Apply consistent diet №4, 4b, 4c. All dishes are steamed or boiled, with normalization of stool baked in the oven. Multiplicity food 5-6 times/day, food is served in the form of heat. Limit the amount of carbohydrates as increasing fermentation, they cause flatulence, abdominal pain, increased stool. Must enter the optimal amount of protein (especially animal - meat, fish), with the loss of protein and disruption of intestinal absorption. The need to ensure the introduction of fats in the diet of butter. At intolerance of any product it is excluded from the diet. Due to the occurrence of secondary changes in mental patients (recess in the disease, narrowing the range of interest, selfishness, negative attitudes towards treatment) need gentle and strictly individual approach to sick children. Effective psychotherapy (rational therapy, suggestion in the waking state, autogenic training) provides confidence in the child's recovery and potentiates the effect of drugs. Drug therapy 18 А. Basic therapy: 1). Preparations 5-aminosalicylic acid preparations are pure 5-ASA (mesalazin, salofalk, Pentas – they are metabolized in the intestine to N-acetyl-5-aminosalicylic acid and inhibit the synthesis of arachidonic acid metabolites, which are mediators of inflammation – level of evidence A) and combined drugs, which, along with 5-ASA includes sulfapiridyn (sulfosalazin, salazopirydazyn, salazodimetoxin – level of evidence C). Most preference is given first, as it provides a therapeutic effect 5-ASA, and side effects are caused mainly sulfapyridine. - Mesalazin - 30-50 mg/kg/day in 3 divided doses. To prevent a recurrence of the disease - 15-30 mg/kg/day in 2 doses. - Salofalk (tablets, granules, suspension rectal, rectal suppositories): older children assigned to 1 tablet 3 times/day after meals for 6-8 weeks with gradual dose reduction. - Pentas (500 mg tablets, granules, suppositories, rectal 1000mg): for of UC in acute children from 2 years appointed by the rate of 20-30 mg/kg/day for 3-4 hours; over 12 years: 1-2 tablets 3-4 times/day, administered candles only older children 1-2 times/day. - Sulfosalazin: daily dose at age 3-5 years = 1.5 g, 5-7 years = 1,5-3 g, 7-15 years = 3-6 g for 3 hours. - Salazopirydazin: daily dose at age 3-5 years = 0.5 g, 5-7 years = 0,75-1 g, 7-15 years = 1,25-1,5 g for 3 hours. - Salazodimetoxin – doses of salazopirydazin. The maximum dose administered to achieve clinical effect, then reduced by ⅓ of the initial 2-3 weeks. If, within 2-3 weeks of the child's condition deteriorated, the daily dose again reduced by ⅓, and as maintenance to retain the completion of treatment in the hospital and continue on an outpatient basis (with mild and moderate form - at least 2-4 months, hard - at least 6 months). If lowering drug comes deterioration, should return to the previous dose. If necessary preparations used in candles and microenema as a 5% suspension salazopirydazin. The effectiveness of this group of drugs is enhanced by the simultaneous appointment of intestinal antiseptics – enteroseptol, intestopan, meksaform, meksaze applicable courses of 7-8 days with an interval of 3-4 weeks, a total of 2- 3 treatments. It is important always to add folic acid preparations. 2). Glucocorticoids: (level of evidence B) - System (prednisone, methylprednisolone, hydrocortisone, dexamethasone, polkortolon): prednisone maximum daily dose – 1-1.5 mg/kg to obtain a therapeutic effect, further decreasing weekly dose of 2.5 mg; maintenance dose – 2.5-5 mg for 1-4 months. - Topical (budesonide, beclomethasone dipropionat, fluticasone dipropionat, tyxokortol): budesonide 3 mg 3 times/day (level of evidence A) Indications of glucocorticoids: . acute course of UC . severe life-threatening form of UC . lack of efficacy of the two-week course of therapy with 5-ASA . intolerance to 5-ASA drugs . system (extraintestinal) manifestations of UC. 3). Ursodeoxycholic acid – 10 mg/kg/day for 3-6 months (level of evidence C). Product: 1 measuring spoon suspension = 250 mg; Tablets of 250 and 500 mg. 4). Systemic Immunosuppressants (azathioprine, methotrexate, 6-mercaptopurine) – azathioprine 1.5-2 mg/kg/day in 2-4 divided doses. Appointed only in specialized hospitals in the absence of efficiency for 2-week hormone therapy (level of evidence A). 5). Hemostatic therapy – menadione sodium bisulfate (0,008-0,015 g / day), 5% solution of aminocapronic acid/drip, 12% solution of sodium etamzylat i/v or i/m 1-2 ml; ½-1 tab 3-4 times/day for 7-14 days (level of evidence B).

B. Symptomatic therapy (level of evidence B) 1) antidiarrheal drugs: - enterosorbents: smectite 1-3 sachets 3 times / day and other - drugs depressing peristalsis: Loperamide 1 capsule 3-5 times / day - spasmolitics (more in "Syndrome of irritable intestinal tract") 2) sedative therapy as needed 3) enzymes that do not contain bile acids - pancreatin and other (individual dosing) 4) antibiotic therapy for joining secondary infection or exacerbation of chronic foci of infection, presence of septic complications and, in particular, antibiotic therapy is indicated for suspected toxic megacolon (semisynthetic penicillins, metronidazole, amikacin, cephalosporins in age doses for 5-7 days). 5) in case of hypochromic anemia - iron supplements at the rate of 1.5 mg / kg / day / m. 6) infusion therapy at the toxicosis eksykozom with the generally accepted rules 7) to normalize intestinal biocenosis at stihanii inflammation - biological products for 3-6 weeks. 8) antifungals excessive growth of fungi of the genus Candida (natamycin 50 mg 2 times / day, 7 days) 9) in case of hypovitaminosis prescribe complex vitamins (ascorbic acid, thiamine, riboflavin, pyridine, retinol) orally or parenterally (assuming expressed hypovitaminosis). 10) to improve the regenerative-reparative processes and microcirculation in the wall of the colon, the therapeutic complex is appropriate to include adaptogens (pentoksil, Methyluracilum inside aloe / m 1 ml daily for 3 weeks), immunomodulators (splenin / m 0.5 -1 ml daily for 7-10 days, with severe forms of AML resistant to glucocorticoids - cyclosporine A) and drugs aimed at normalization of the microcirculation (intensayin ½-1 tab 3 times / day for 3 weeks). 19

В. Surgical treatment - total kolonektomiya. Indications for surgical treatment: - severe colitis in children 1 year of age - moderate and severe colitis with no effect on basal drug therapy - complications UC - fulminant course UC. The general scheme of destination therapy depending on the severity UC Severity Scheme prescriptions Easy 5-ASA drugs (mesalazin) at the distal location - in the candlelight, microclysters or a combination of oral and rectal route of administration; sulfasalazin or orally, chelators, biological products, multivitamins. Moderate 5-ASA drugs (mesalazin), with no effect - corticosteroids (topical, with no effect - systemic oral), infusion therapy, anti-anemic, hemostatic agents, antispasmodics, biologics. Severe Glucocorticoids (orally, if necessary - in / in), with no effect - combined with Immunosuppressants; infusion therapy, transfusion of blood components, chelators, hemostatic agents, anti-anemic drugs, biologics. If no effect - surgery (total kolonektomiya). Duration of hospital treatment - individually to obtain a therapeutic effect. Requirements for the results of treatment - removal hemocolitis, achieving long-term clinical and laboratory remission. Prevention Prevention is aimed primarily at preventing relapse. After hospital discharge all patients should be advised courses and supporting anti-treatment, which includes basic medication, diet, and security and recovery mode. Patients with AML are always under medical supervision. Children exempt from exams, physical activity (physical education, labor camps, etc..). Study preferably carried out at home. Preventive vaccinations and spa treatments are contraindicated (only epidemiological indications attenuated preparations). Shows regular monitoring changes in the mucous membrane of the colon because of the possibility of dysplasia. Most experts recommend using acetaminophen for pain relief rather than non-steroidal anti-inflammatory drugs such as ibuprofen and naproxen. Clinical supervision - is carried throughout life.  antirecurrent treatment - 2 times / year in hospital  review of pediatric gastroenterologists and pediatric surgeon - 2 times / year  review pediatrician - per month (first 3 months after discharge from the hospital, then every 3 months)  review by other experts - on request  CBC: 2 times / month (first 3 months), then - per month (for a year), then - 1 time / 3 months  proteinogram, biochemical indices of Liver’s function, coagulation, acute phase indicators - 1 times / 3 months (first 2 years), then 1 time / year  urinalysis, coprogram - 1 times / 3 months (first 2 years), then 1-2 times / year  fecal helminthes eggs, protozoa - 2 times / year  fecal bacteria overgrowth - 2-3 times / year  fecal (reaction Gregersen, Tribule) - 1 times / 3 months (first year), then - 1 time / year  ultrasound of abdominal cavity organs - on request  sigmoidoscopy - 1 times / 3 months (first year of surveillance), then - 2 times / year  irrigography - 1 times / year. Prognosis The prognosis for recovery is poor for life - depends on the severity of illness, disease, presence of complications.

CROHN'S DISEASE Crohn's disease (HC) (sin.: terminal, of regional ileitis, granulomatous enterocolitis) – code K 50 - a chronic relapsing disease characterized by transmural granulomatous inflammation, ulcerative mostly terminal part of ileum and cecum. The etiology of the disease is unknown. Incidence of Crohn's disease 3.5 100 000 population. Pathogenesis In the pathogenesis of essential genetic factors are primarily genetically determined abnormalities of function of neutrophilic granulocytes that when HC are lower than in healthy, phagocytic activity. Have some connection with genetically caused disease markers - major histocompatibility complex antigens HLA, antigens are often HLA DR1, DRw5, specific clones of B-lymphocytes and V134 Villa. It is believed that when there is a specific HC associated only with the disease antigen that has determinants of native intestinal epithelium. Autoimmune processes in HC become pathological and cause severe systemic and local inflammatory changes in the intestinal mucosa. A characteristic feature of HC is a focal lesion with clear demarcation from unaffected areas. Hyperemic, hilly, moderate bleeding areas alternating with intact mucosa. Initially there lesion of the submucosal layer, gradually involved in muscle membrane and subserous layer. Is marked thickening and swelling of the intestinal wall, the formation of 20 granulomas with epithelioid cells without caseous basis, affected lymph vessels. As a result, the intestinal lumen is narrowed, fistula formation, adhesions, ulcers. Classification of CD (V.N.Kopyeykin et al., 2001) Form Localization Ileitis, ileokolit, anorectal, gastric, 12 duodenal ulcer, other localization Period Infiltration, cracks, scarring, stenosis Phase Exacerbation Severity: mild, moderate, severe Remission Course of the Acute, subacute, chronic disease Extraintestinal manifestations: spondylitis, peripheral arthritis, aphthous stomatitis, erythema nodosum, uveitis, scleroconjunctivitis, hepatitis Complications: fistula (intercolon, perineal, etc.), perianal lesions, intestinal stenosis, obstructive ileus, bowel perforation and peritonitis, intestinal bleeding, toxic megacolon, amyloidosis, toxic-septic conditions, vascular thrombosis, thromboembolism Concomitant diseases Clinic Clinical symptoms of HC have much in common with symptoms of ulcerative colitis, however, depending on the location, length of lesion, duration of illness have their own characteristics. The disease often occurs gradually, but steadily progressive, relapsing course characterized by undulating, accompanied by abdominal pain, diarrhea, presence of blood, pus or mucus in the stool, fever, progressive weight loss, anemia patient. Acute terminal ileitis forms are rarely identified. For HC the colon characterized by severe diarrhea, which often precedes hemocolitis, paroxysmal abdominal pain during defecation, fecal contaminants to a large number of pus and dark blood (from the proximal colon). Emptying the stinking, frothy, with a lot of fat, calories like floating in the water. Because diarrhea is disturbed absorption of vitamins, electrolytes. In some patients the main clinical symptoms are lesions anus plots shown itching of the skin, formation of deep cracks sphyncteritis, paraproctitis, fistula, abscess. As a result of the formation in the intestinal wall slit deep ulcers often formed intestinal fistulas, covered by punching, intercolon adhesions with the appearance of infiltrates in the abdomen. Review On examination the child was observed: • Weight loss, sometimes - growth retardation and sexual development. Determine the height and weight according percentile. Deviations of these parameters below the third percentile assist in determining the severity of the disease. • Oral examination (presence of AFL, gingivitis) • may be affected eye (uveitis, iritis, conjunctivitis) • changes in the skin and joints (erythema nodosum, pyoderma gangrenosum, arthritis, spondylitis, sacroileitis, signs of hypovitaminosis). • palpation of the abdomen - abdomen is soft, swollen, painful around the umbilicus and in the right inguinal area. Can palpate infiltrate in the right lower quadrant of the abdomen; compaction segmental colon; perianal fissures; increase in liver size (in severe forms of the disease). Regardless of the location of the pathological process in HC there are common disorders: fever (from subfebrile to hectic temperature), weight loss, signs of anemia, polihypovitaminosis, electrolyte hypoproteinemic failure and edema. Characteristics severity CD depending on the localization of the pathological process and the presence of complications Mild disease: - Loss of appetite - Abdominal pain, flatulence occasionally, - Unstable stool (constipation vary mushy feces). Moderate degree of severity: - Loss of appetite - Pain attacks, strong, after eating may be aching, constant (localization depends on the largest lesion) - Feeling transfusion, meteorizm, bloating - Diarrhea (semi mushy stool up to 10 times / day mixed with mucus, pus, blood) - Palpation: meteorizm, pain around the navel, noise, clapping, painful dense section of the affected bowel. Severe degree of severity: - anorexia - paroxysmal pain, severe, perhaps permanent, reinforced during the journey, after meals and before the act of defecation - manifestations of intestinal dyspepsia violation stool (semi mushy stools 10-12 times / day mixed with mucus, pus, blood), tenesmus, urgency to defecate at night - palpation pain at the site of bowel lesions may be tumor formation (if intercolon adhesions). Pediatric Crohn's disease activity index (PCDAI) Symptoms Criteria Points Abdominal pain Absent 0 A slight intensity 5 considerable intensity 10 Stool, frequency, consistency 1 per day, liquid, without impurities of blood 0 21 2-5 times a day with a small amount of blood 5 More than 6 times a day 10 The state of health, activity No limitation of activity 0 Moderate activity restriction 5 A significant limitation of activity 10 Body weight No weight loss 0 Weight loss of 1-9% 5 Weight loss> 10% 10 Height Below 1 percentile 0 From 1-2 percentiles 5 The following 2 percentiles 10 Palpation tenderness No pain 0 Pain, seal intestine 5 Expressive pain 10 Perianal signs Absent 0 Fistula, abscess 10 Extraintestinal manifestations Absent 0 One 5 More than two 10 Hematocrit in children under 10 years, % > 33 0 28-32 2,5 < 28 5 Hematocrit (girls) 11-18 years, % > 34 0 29-34 2,5 < 29 5 Hematocrit (boys), 11-14 years, % > 35 0 30-34 2,5 < 30 5 Hematocrit (boys) 15-18, % > 37 0 32-36 2,5 < 32 5 ESR mm/h < 20 0 20-50 2,5 > 50 5 Albumins g/dL < 3,5 0 3,1-3,4 5 > 3,0 10 Notes: <10 points - no activity (remission); 11-30 points - light or medium heavy degree; 30-100 points - severe degree of the disease. Diagnosis  World experience shows that early diagnosis of HC reduces the risk of complications (level of evidence B).  Required study:  CBC (anemia, leukocytosis, accelerated erythrocyte sedimentation rate)  ZAS, testing for worms (feces and blood)  Biochemical blood parameters (total protein and fractions - dysproteinemia with hypoalbuminemia, hyper-alpha 2- globulinemiya, total bilirubin and fractions, electrolytes - reduction of K, Fe, Mg, Zn, folic acid transaminase)  Acute phase inflammatory blood parameters (CRP, seromucoid, sialic acid) - in acute elevated  Koprotsytohrama (steatorrhea, positive test Triboulet with chlorine and trichloroacetic acid, positive test Gregersen)  Fecal bacteria overgrowth (available dysbiotic change)  ! The diagnosis of HC verified according to endoscopic, radiological and morphological studies.  R-graphy gastrointestinal (irrygography): values depend on the phase of the disease and are divided into non- stenotic (early) and stenotic symptoms  Non-stenotic symptoms are intermittent appearance, characterized by rigidity of affected areas, mosaic images due to edema, linear ulcers, svyschovyh moves contrast penetration beyond the bowel wall in a pocket or fringe  continue to appear stenotic symptoms: reduced or disappears completely contractile ability intestine slows evacuation of barium, there is irregular narrowing of the intestine to the state of "lace", while outside there is a significant narrowing of its expansion. - Endoscopic examination (sigmoidoscopy, colonoscopy, esophagogastroduodenoscopy) morphological study of gastric biopsies, 12 duodenal ulcer, intestinal. During endoscopy, colonoscopy is the method of choice that allows you to examine the entire colon, with right- sided localization process sigmoidoscopy may be ineffective due to the lack of changes in the rectum. Picture polymorphic and depends on the phase of disease (infiltration, destructive, scarring). For lesion length process can be local and diffuse. 22 Endoscopic characteristics of Crohn's disease  phase infiltration: lesions deeper layers of the wall (luminal narrowing, mucosal edema, vascular pattern is determined only by large vessels may be small aphthous defects).  split phase (destructive): large ulcer defects in the form of longitudinal cracks along with direction or transverse colon, relief in the form of "paper pavers."  phase scarring: cicatricial stenoses that cause intestinal obstruction. Histological examination of biopsy: presence of longitudinally spaced ulcers, cracks that penetrate the muscular and subserous layers granulomas tuberkuloyidnoho and sarkoyidnoho types of cells Pirogov-Langhans in the submucosal layer.

Symptom "Bridge" Linear ulcers on the background of unchanged mucosa

Schematic structure of the X-ray pattern in Crohn's disease

Cecum

Sleek ulcerogenic segment ileum

Pseudodiverticul

Stricture

During the differential diagnosis, other than ulcerative colitis should be excluded, especially acute appendicitis (no signs of pathological process in the intestine, diarrhea, hemocolitis) and acute infectious diseases (dysentery, salmonellosis, shigellosis). Differential diagnosis of ulcerative colitis and Crohn's disease Indicators Ulcerative Colitis Crohn's Disease Acute course Often rarely Fulminate course Occurs No Clinical sighs Intestinal bleeding Regularly Occasionally, with involvement of the rectum Diarrhea Night, expressed 4-6 times during the day Constipation Sometimes, when proctitis Characteristic Pain in the abdomen Minor symptoms associated with defecation Intensive Infiltration Absent Often projection rectum Perforations Against the backdrop of a toxic bowel dilatation Hidden perforation in the abdominal cavity 23 Internal fistula Not found Frequently Surface fistula Not found The skin of the anterior abdominal wall Intestinal obstruction Not found Often, typical for ileocolitis Clinical remission It may be partial or complete Complete remission is not Malignant degeneration Frequency increases with the total defeat and Sometimes duration of disease more than 10 years Characteristic of Same features as in early disease Relapses are frequent, but without them, exacerbations patients do not feel healthy Defeat anal area 20% excoriation of the skin or some anal fissures 75% - abscesses, fissures, ulcers Prevalence process It starts from the rectum Segmental nature of the lesion, often - the ileocecal area Stricture Atypical, suspicious for malignancy Often. In the terminal ileum Recurrences after surgery No In 50% of cases Radiographic signs The defeat of the rectum Permanent In 50% of cases, often with fistula Narrowing of the lumen Even without clogging Local stricture, intestinal obstruction of the intestine The evacuation of barium Normal or accelerated Weakened Intestinal wall Elastic Rigid View mucosa Evenly granular with psevdopolipamy Looks like "bridge" Ulcers Multiple In the form of deep cracks Shortening of colon Often gives rise to symptoms of "water pipe" Segmental, often with fistula Intestinal lumen Diffuse narrowed Symptom "cord" Endoscopic signs of ulcerative colitis and Crohn's disease Symptom Ulcerative Colitis Crohn's Disease Prevalence of lesions widespread, continuous, affected the entire limited, segmental, focal circumference of the bowel Involvement of the distal colon always less than 40% Lesions of the ileum occasionally 50-70% Serosa not affected thickened fibrous The transition process in the there is no possible mesentery Bowel stricture not typical characteristic The mucous membrane of the bright diffusely hyperemic, edematous hyperemic, bumpy colon Ulcers surface, irregular shape, may merge aphthous, deep, longitudinal coupled with cross fissuramy Contact bleeding present absent Pseudopolyps typical not typical The thickness of walls slightly thickened greatly thickened Spontaneous fistula not typical often Anal fistulas and fissures occasionally often Malignization possible not typical Histological features of ulcerative colitis and Crohn's disease Symptom Ulcerative Colitis Crohn's Disease Inflammation involves тільки слизову оболонку усі шари Submucosal layer поверхневий фіброз, глибокий фіброз васкуляризація Lymphoid hyperplasia слизової оболонки усіх, шарів, гістіоцитарна інфільтрація Epithelial-cell granulomas with absent present in 75% Langhans cells in the submucosal layer Fissury no present, is transmural Cryptogenic abscesses present absent Pseudopolyps often occasionally Changes vessels vasculitis lymphangitis, lymphostasis, obliteration of arterioles Regional lymph nodes nonspecific reactive hyperplasia granulomas in 50% Treatment The goal - to achieve remission, prevention of complications, reduction of physical and psychosocial development according to age. Treatment of children with ChC is virtually indistinguishable from that in ulcerative colitis. In acute diseases recommend hungry pause for 24-48 h with simultaneous parenteral nutrition and subsequent gradual transition to a diet №4. 24 І. Diet therapy - confirmed by clinical practice [The second European evidence-based Consensus on the diagnosis and management of Crohn's disease: special situation, 2010] (level of evidence C) Fulminant form With malabsorption syndrome: А. Full enteral nutrition - a specialized blend from whey protein hydrolyzate (eg. Peptamen) through a tube or orally. Calculation of dose mixture is based on the daily needs of the child to the existing body weight (kcal/kg/day). On the first day the patient should receive no more than 50% of the required daily intake. With adequate tolerability mixture on the second day the dose is increased to 75% on the third day of 100%. B. Probe enteral nutrition mix - drip or spray no more than 200 ml per hour, or orally - 100-200 ml for 30-40 minutes in small sips. No malabsorption syndrome: А. Full enteral nutrition mixture containing transforming growth factor (eg .. Modulen IBD) through a tube or orally. Calculation of dose mixture is based on the daily needs of the child to the existing body weight (kcal / kg / day). In the first day the patient receives less than 50% of the required daily calorie content. With adequate tolerability mixture on the second day the dose is increased to 75% on the third day of 100%. B. Probe enteral nutrition - regime of administration: infusion or bolus no more than 200 ml per hour. Orally: 100-200 ml mixture for 30-40 minutes in small sips. Severe form of the disease In cases of lesions of the small intestine and the presence of malabsorption syndrome mixture of first-line of full enteral nutrition is a specialized blend from whey protein hydrolyzate with subsequent transition to a mixture containing transforming growth factor. Mixture from whey protein hydrolyzate used either as a sole source of nutrition, and the mixture is calculated dose according to body weight (kcal/kg/day), or as an auxiliary power source at a dose of 400-600 ml per day. Any damage to the colon daily ration child is completely replaced by a mixture containing transforming growth factor. Calculation of dose mixtures made in view of the daily needs of the child, due to body weight (kcal/kg/day). Time between doses mixture should not exceed 1.5 hours. With adequate tolerability reception mixture lasts for 8 weeks. After that, the child expands diet (treatment tables №5p, 5), but can still use the mixture at a dose of 400-600 ml / day for 4 weeks in the future - a dose of 200-400 ml per day to obtain sustained clinical and morphological remission . Moderate form of the disease Treatment tables №4-5p-5. Appointment mixture containing transforming growth factor as an auxiliary supply, the calculation of the dose, mode of mix and duration of its use - as in severe disease. Mild form of the disease Enteral nutrition is carried out with a mixture containing transforming growth factor as an auxiliary power source at a dose of 400-800 ml/day. Dose calculation is performed taking into account the daily needs of the child, due to body weight (kcal/kg/day). The mixture should be consumed within 30-40 minutes in small sips. ! Mixes from whey protein hydrolyzate and containing transforming growth factor used in school-age children and adolescents (level of evidence B). In remission - treatment table №5 (confirmed by clinical practice - level of evidence D). ІІ. Drug therapy International experience treating children proves that the pathogenetic drug therapy promotes rapid elimination of clinical manifestations of the disease, reduce the term of hospitalization (level of evidence B) General recommendations: • 5- aminosalicylic acid (5-ASA): – mesalazin 50-60 mg/kg/day, the maximum dose of 4.5 g per day (level of evidence A); – salofalk (tablets, granules, suspension rectal, rectal suppositories): children 2-12 years during the exacerbation rate of 30-50 mg/kg/day, teenagers - 3,0-4,5 g/day; in remission - children 2-12 years 15-30 mg/kg/day, teenagers - 1.0-1.5 g/day (level of evidence A); – Pentas (tablets, granules, suppositories, rectal): children 2-12 years in acute assigned a rate of 30-50 mg/kg/day, teenagers - 1.5-4 g/day; in remission - children 2-12 years 15-30 mg/kg/day, teenagers - 2 g/day; • glucocorticoids show patients that have no effect on the use of 5-ASA drugs; patients with lesions of the gastrointestinal tract (from esophagus to jejunum) and / or with extraintestinal manifestations (level of evidence B). Topical glucocorticoid budesonide shown patients with mild and moderate forms of the disease in acute and in patients with lesions of the distal ileum and ascending colon; The optimal dose of budesonide was 9 mg per day (level of evidence A); • cytotoxic agents (azathioprine or its active metabolite 6-mercaptopurine) shows hormone resistant patients or where necessary to reduce the dose glucocorticoids due to the presence of adverse reactions (level of evidence A). The recommended dose is azathioprine 2.5 mg/kg/day, 6-mercaptopurine - 1-1.5 mg/kg/day. Contraindications to the appointment of azathioprine is the presence of pancreatitis. • antibiotic therapy: metronidazole 20 mg/kg/day in patients with primary localization lesions in the colon (level of evidence A); Antibiotics, Antifungal drugs - see. "Ulcerative colitis"; • monoclonal antibody to tumor necrosis factor α: infliximab is recommended for / in use in children of 6 years with severe and moderate in the form of a single dose of 5 mg / kg. Side effects - headache, dyspnea, rash, infections such as tuberculosis. Variants of pathogenetic therapy depending on the severity of the disease

25 Experience of treatment of children in the world proves that the pathogenetic drug therapy eliminates most clinical manifestations of the disease, reduces the length of hospitalization (level of evidence B) Fulminant disease (fever, vomiting, diarrhea, intestinal bleeding, muscle protection palpation of the abdomen, accelerated ESR, increasing the total protein content, dysproteinemia, anemia): - prednisolone or methylprednisolone 1-1.5 mg/kg/day + azathioprine 1.5-2 mg/kg/day. A positive response - reducing the dose of glucocorticoids (2.5 mg every 7-10 days), azathioprine - continue at the same dose. Achievement of remission - maintenance therapy azathioprine 1.5-2 mg / kg / day for 2 years. If no effect - infliximab 5 mg / kg (w / 2 hours) scheme 0-2-6 weeks; future - every 8 weeks to achieve remission. Achievement of remission - infliximab maintenance therapy at a dose of 5 mg / kg every 8 weeks for 1 year and azathioprine at a dose of 1.5-2 mg / kg for at least 2 years. The lack of effect in the future - surgical treatment. - infusion replacement therapy - electrolyte solutions, if necessary – transfusion substitutes products. Disease activity from moderate to high - prednisone i/v 2 mg/kg/day. In the absence of effect of prednisone for 7 days administered cytotoxic agents - azathioprine 1.5-2 mg/kg/day (100 mg/day), while achieving remission - azathioprine maintenance therapy at a dose of 1.5-2 mg/kg/day did not less than 2 years. Disease activity from mild to moderate - with lesions of the distal ileum prescribe 5-ASA (mesalazane 50 mg/kg/day, the maximum dose - 4.5 g/day); - if the lesion is limited to the ileocecal area and ascending colon - budesonide 9 mg/day. Maintenance therapy mesalazane at a dose of 15-30 mg/kg/day for at least 1 year. Children with CD requiring surgery in case of complications (level of evidence C) - intestinal bleeding - intestinal obstruction with signs of obstruction, abscesses, fistula - refractoriness to drug therapy, lag in the physical development of children Quality criteria of treatment: - no clinical signs of disease, long-term clinical and endoscopic remission - absence of complications, compliance levels of physical and psychosocial development of the child's age. Clinical supervision of children with CD - for life. Analogous to surveillance in ulcerative colitis. Sanatorium treatment at CD - not shown.

6. The materials for self-control A. Sample case report A 8-year-old boy presents with a history of abdominal discomfort of 11 month’s duration. The discomfort is located in the right, left, and mid-upper abdomen. There is no association of the pain with activity, position, meals or the act of defecation. The abdominal discomfort occurs more commonly at night. The pain may last as long as 4 hours. He often experiences nausea when he has the pain and vomits at least once a week. Bowel movements occur once a day and are not related to pain. 1.What is the most likely diagnosis? - Chronic non-ulcer colitis. 2. Prescribe treatment.

B. The tests for self-control 1. A 14 y.o. boy has been suffering from nonspecific ulcerative colitis during 5 years. On rectoromanoscopy: marked inflammatory process of lower intestinal parts, pseudopolyposive changes of mucous. In blood: WBC – 9, 8 * 109/L, RBC – 3, 0 * 1012/L, erythrocyte sedimentation rate – 52 mm/hour. What medication provides pathogenetic treatment of this patient? Sulfasalasine Motilium Vikasolum Linex Kreon

2.A 8 y.o. boy complains of stomach pain, which relieves with defecation, and is accompanied by flatulence, rumbling, the feeling of incomplete evacuation, constipation or diarrhea in alternation. These symptoms have lasted for over 3 months. No changes in laboratory tests. What is the most likely diagnosis? Irritable bowel syndrome Spastic colitis Colitis with hypertonic type dyskinesia Chronic enterocolitis Atonic colitis

3. A mother of a 5-year-old boy has been noting the presence of mucus and blood in his feces during last two months. A liquid stool 3-4 times per day is observed. The general condition of the child is not disturbed. Body temperature is normal. Normal appetite, he has not put off weight. Objectively: abdomen is soft, a little bit painful in the left iliac region on palpation. What diagnostic method should be conducted to confirm the diagnosis? Endoscopic examinations Ultrasound of abdomen 26 X-ray of the intestine Bacteriological examination of feces Biochemical analysis of blood

4.A mother of a 4-year-old boy has been noting the presence of mucus and blood in his feces during last month. A liquid stool 4-5 times per day is observed. The general condition of the child is not disturbed. Body temperature is normal. Rektoronomanoskopi revealed single slight ulcers and erosions, coated with fibrin on colon mucosa. What is the most likely diagnosis? Nonspecific ulcerative colitis Acute dysentery Campylobacteriosis Crohn's disease Irritable Bowel Syndrome

5.A 14 year-old child suffering from ulcerative colitis suddenly developed abdominal pain, vomiting, lack of defecation. On the X-ray of the abdominal cavity a significant extension the intestinal area is observed. What complication of the disease caused this condition? Toxic dilation of the colon Intestinal perforation Intestinal bleeding Intestinal polyposis Intussusception

6.A 13-year-old boy was admitted to a hospital with complaints of diarrhea to 3 times a day mixed with blood in the stool, moderate cramping pain in the left abdomen, low-grade fever, loss of appetite. Objectively: pale skin, retarded body weight. Abdomen is soft, palpation reveals a painfulness of the left parts of the colon. In blood: Hp-90 g/L, erythrocyte sedimentation rate – 25 mm/h. After an examination the patient was diagnosed with ulcerative colitis. What therapy is advisable in this case? 5-ASA drugs Cytostatics Antibiotics Cardiac glycosides Corticosteroids

7. Literature Basic: 1. Pediatrics: Textbook for students of higher medical educational institutions III-IV levels of accreditation. / VG Maidannik. - Kharkov: Folio, 2006. 2. Children's Diseases (V.M Sidelnykov, V. Berezhnaya, B.J. Resnik, etc.). - K.: "Health", 1999. 3. Medical childhood / gen.ed.prof. Moschych S.K.: Health, 1994 Additional 1. Kliegman: Nelson Textbook of Pediatrics, 18th ed. Copyright © 2007 Saunders, An Imprint of Elsevier / Chapter 333 – Inflammatory Bowel Disease/ Jeffrey S. Hyams. 2. Kliegman: Nelson Textbook of Pediatrics, 18th ed. Copyright © 2007 Saunders, An Imprint of Elsevier / Chapter 335 – Disorders of Malabsorption/ Manu R. Sood. 3. Apley J. The child with abdominal pains. 2d ed. Oxford: Blackwell Scientific, 1975. 4. FUNCTIONAL ABDOMINAL PAIN SYNDROME R.E. Clouse, E.A. Mayer, Q. Aziz, D.A. Drossman, D. Dumitrascu, H. Monnikes, B.D. Naliboff // Римський консенсус III, 2006.

The methodical instructions are compiled by Bubyr L.N.

Methodical instructions were discussed and approved at the meeting of department of Pediatrics №2 protocol № ___ of ______20____.

Additions and changes were made “____” ______20____ Additions and changes were made “____” ______20____ Additions and changes were made “____” ______20____ Additions and changes were made “____” ______20____ Additions and changes were made “____” ______20____

27 MINISTRY OF HEALTH OF UKRAINE UKRAINIAN MEDICAL STOMATOLOGICAL ACADEMY

“APPROVED” at the meeting of department of Pediatric № 2 “____05__” ______06_____ 2020 Protocol № 21 Head of the department MD, Prof. Kryuchko T.O. ______

Methodical instructions for independent work of students during preparation to practical classes and in class Academic discipline Pediatrics

Module # 6 Topic of the lesson Urinary system infections in children. Dysmethabolic nephropathy in children. Course 4 Faculty Medical faculty

Poltava 2020 1. Relevance of the topic: Problem of the infections of urinary tract in children is the most relevant in children’s nephrology, cause of this group of the diseases takes the first place in structure of nephropathies, significantly exceed wide spreading of glomerulonephritis and other nephropathies. In Ukraine wide spreading of the diseases of urinary organs in children is rising up during last 5 years, due to nephrological diseases increasing of the kidneys infections and urinary tract infections are signed In structure of nephrological pathology pyelonephritis takes the 10 – 15% of all cases.

2. The specific aims: − to estimate etiological and pathogenesis factors of urinary tract infections (cystitis, pyelonephritis), dysmethabolic nephropathy in children; − to estimate main etiopathogenic reasons that influence development of acute and chronicle forms of cystitis and pyelonephritis; − to classify and analyze typical clinical picture of the infections of urinary tract (cystitis, pyelonephritis), dysmethabolic nephropathy in children; − to estimate peculiarities of the urinary system in newborns and infants; − to create plan of examination and analyze results of laboratory and instrumental investigations in case of urinary tract infections (cystitis, pyelonephritis), dysmethabolic nephropathy in children: general clinical and biochemical blood tests, general urinary test, methods of quantity estimation of blood components and proteinuria, estimation of bacteriuria, fractional cystographia and excretory urographia, echographia, radiological exploration, nephrobiopunction; − to provide differential diagnostic and put previous diagnose in case of urinary tract infections in children; − to demonstrate skills of treatment, rehabilitation and prevention of urinary tract infections (cystitis, pyelonephritis), in children; − to demonstrate skills of ethical and deontological principles of medical worker and principles of subordination in children’s nephrology 3. Basic knowledge, skills, habits necessary for studing of the topic (inter-disciplinary integration) The names of the preceding disciplines The acquired skills Normal anatomy, Physiology Anatomic and physiologic features of the urinary system in children. Pathologic physiology Pathophysiologic mechanisms of urinary system infections and dysmethabolic nephropathy. Determine the etiology of urinary system infections and dysmethabolic nephropathy based on knowledge of pathophysiologic mechanisms. Biochemistry To give a clinical assessment of changes in biochemical parameters of blood and urine Pharmacology and clinical pharmacology Determine the indications for therapy, calculate the dose of drugs, prescribe prescriptions. Propedeutics of pediatric diseases Physical examination of the urinary system in children. Perform physical examination of urinary system (gross inspection, palpation, percussion).

4. Tasks for independent work in preparation for class and in class. 4.1. List of the main terms and characteristics to know during the preparation to practice: Definition Meaning Its meaning concerning of the infection in organs of urinary tract without knowledge about its localization; in case of such condition we don’t have results concerning impression of the parenchyma 1. Infections of urinary tract tissue of kidneys, but signs of transitory inflammation of low urinary ways, estimate concrete localization in time of examination is impossible Non specific microbial-inflammatory disease of kidneys with advantage focal inflectional inflammatory damage of 2. Pyelonephritis tubule-interstitional tissue, concerning with infection of urinary tract, that gets to kidneys with hematological, lymphatic or upper ways 3. Cystitis Inflammation of the mucous layer of the cystic Condition, in background of which combination of dysembriogenesis of urinary tract and some elements of nervous system. Is 4. Reflux - nephropathy characterized with development of chronicle pyelonephritis at background of cystic-urethral reflux Group of urological diseases, that are supervised with violation of urinary circulation and increasing of intrapelvical pressure, 5. Obstructive uropathies spreading of cup-pelvical segments, development of secondary pyelonephritis with gradually atrophy of parenchyma tissue Quantity and quality features, get during exploration of urine and 6.Urinary syndrome process of secretion 4.2. Theoretical questions for the practice: 1. The etiology of infections of urinary tract. Risk factors of development of IUT in children’s age. 2. Pathogenesis, pathomorphology of acute pyelonephritis, cystitis. 3. Meaning of the obstructive uropathies (organical and functional) in development of secondary and chronic forms. 4. Clinical classification of cystitis, pyelonephritis in children and principals of the diagnose estimation according to it. 5. Clinical signs of cystitis and pyelonephritis in children. 6. Principles of the diagnostic of cystitis, pyelonephritis (anamnesis, clinical and laboratory- instrumental results). 7. Differential diagnosis of pyelonephritis: - acute and chronic glomerulonephritis; - interstitional nephritis; - dysmetabolic nephropathies; - toxic nephropathies; - cystitis, vulvovaginitis, urethritis. 8. Principles of treatment of IUT (cystitis, pyelonephritis). 9. Prevention of development of IUT, principles of preventive antirelapse therapy. 10. Pathogenesis, pathomorphology of dysmethabolic nephropathy 11. Clinical classification of dysmethabolic nephropathy in children and principals of the diagnose estimation according to it. 12. Clinical signs of dysmethabolic nephropathy in children. 13. Principles of treatment of Prevention of development of dysmethabolic nephropathy.

4.3. Practical skills made in practices: 1. Work with test tasks. 2. Work in wards with children with cystitis, pyelonephritis.

3 3. Interpretation of additional methods of the examination. 4. Clinical analysis of typical patient. 5. Solving of situational tasks.

Content of the topic. Pyelonephritis – is non specific microbial-inflammatory disease of kidneys with advantage focal inflectional-inflammatory damage of tubule-interstitional tissue, concerning with infection of urinary tract, that gets to kidneys with hematological, lymphatic or upper ways. Etiology. 1. Escherichia coli 2. Proteus 3. Enterococcus 4. Pseudomonas aeruginosa 5. Staphylococcus 6. Bacterial associations 7. Others: Morganella morganii, klebsiella oxytoca, Citrobacter freundii, Streptococcus pyogene, Candida albicus. 2. Classification of pyelonephritis. Form Currency Activity Kidney function Primary (non Acute Active period Saved obstructive) Chronic Part clinic Damaged

laboratory Secondary a) relapsing Chronic kidney remission (obstructive) b) latent insufficiency Full remission Classification of cystitis: 1. Form: primary, secondary. 2. Etiology: bacterial, fungal, viral, medicine-reduced. 3. Localization: local, diffused. 4. Morphological signs: catarrhal, hemorrhagic, granulated, necrotic, interstitional. 5. Currency: acute, chronic (relapsing, latent). 6. Complications: cystic-uretheral reflux, pyelonephritis, enuresis. Diagnostical clinical criteria of cystitis: 1. Dysurietic changes. frequent painful urinations, that supervised: - painful urination calls and urine unkeeping; - reflector spasm of cystic sphincter and perineum muscles as result of acute pain, that leads to delay of urination. 2. Pain syndrome. Abdominal pains, at pubical region, caused with full cystic and spreading of its borders, irradiation of the pain to the perineum region, anus. Palpation of cystic is acutely painful, as result of increased sensitivity of inflammated mucous layer and tonic muscle movements. 3. Intoxication syndrome. Not manifested. Increasing of the symptoms concerning with development of pyelonephritis. Diagnostical laboratory-instrumental criteria of cystitis: 1. Urinary syndrome: severe leucocyteuria of neutrophilic type, bacterialuria, erythrocyteuria (fresh erythrocytes), unsignificant proteinuria (postrenal). 2. Haemogramm: without changes, possible small leucocytosis, erythrocytes sedimentation rate up to 15 – 20 mm/h 3. Bacteriological test of urine: bacteriouria of different stage of severity 4. USS: thickening of the cystic wall, possible salt content. Diagnostical clinical criteria of pyelonephritis: 1. Pain syndrome: abdominal pain or at region of the back.

4 2. Intoxication syndrome: (rising of the body temperature, pale skin, periorbital cyanosis, vomiting, bleeding). Main possible clinical signs: - beginning: acute or progressive with hidden period - preceding in 7 – 21 day infection disease, stress - in newborns – bad increasing of body weight, cyanosis of skin, anorexia, dyspeptic signs, excitement, long-lasting jaundice, tetany - dehidratation signs: decreasing of skin turgor, dryness, tachycardia, thirstiness - intestinal syndrome - disorder of urination rhythm: rare or frequent urination, delay of urination, unkeeping of urine in day- or night-time - cloudy urine - anamnestic results concerning abnormalities of development of urinary system in relatives - for girls – relapsing vulvitis, vulvovaginitis, enterobiosis Diagnostical laboratory-instrumental criteria of pyelonephritis: 1. Changes in blood test. 2. Urinary syndrome. 3. Instrumental examinations. The interpretation of a persistent crystalluria must be done according to the clinic. Drug crystals are sometimes found in urine. In most cases, these findings are of little clinical value except, if the sediment's picture indicates a possible renal obstruction. Crystal casts are pathognomonic of this situation. Some think that giving much time to the identification of unusual crystals is not worthwhile. Crystals related to urolithiasis are, except for cystine, usual and easy to identify. Calcium is found in 80 to 95% of kidney stones, mostly as oxalate or phosphate crystals. Many stones are not homogeneous. Some have a nucleus of a different composition from the surrounding matrix. Type Occurence in % Calcium oxalate Whewellite (mono-hydrate) 70 Weddellite (di-hydrate) Calcium phosphate Hydroxyl-apatite Carbonate-apatite 10 Calcium hydrogen phosphate (Brushite) Tri-calcium phosphate ( Whitlockite ) Triple phosphates (Magnesium ammonium phosphate) 5 to 10 (Struvite) Uric acid <5 Cystine 1

Hypercalciuria An increased urinary calcium elimination can result in a crystalluria, mostly as calcium oxalates. The superior limit for the calciuria is 75 mmol/d under a 250 mmol/d diet. Hypercalciuria can be caused by: • an increase of the fraction of the diet absorbed. • a renal loss with a secondary increase in intestinal absorbtion. • an excessive bone resorbtion. • a primary hyperparathyroidia. • a combination of the previous causes.

Hyperoxaluria The calcium oxalate is probably the crystal that one meets the most frequently in a urinary

5 sediment. In the majority of cases, the presence of these crystals is without any clinical meaning. According to Conyers, only 10 to 15% of the urinary oxalate is directly related to the diet. The majority of the urinary oxalates is produced by the metabolism (glyoxilic acid cycle). It seems that even light hyperoxaluria is, after the decreased urinary volume, the most significant factor in the recurrent calcium oxalate urolithiasis. In some cases, the crystallization of the calcium oxalate is massive and catastrophic. A typical example of the oxalate clinical catastrophe is the cases of ethylene glycol poisoning. In this situation, one can find oxalate crystals in the patient's tissues. The toxicity syndrome affects organs like the liver, the kidney, and the brain and is accompanied by a metabolic acidosis. Naturally, the oxalate crystalluria is massive, and is predominated by the ovoid crystals (Whewellite) forming microlithes. Hyperuricosuria and calcium oxalates An increased elimination of urate is most frequently caused by a high purine diet. Overproduction, can also be a cause of hyperuricosuria. With a urinary pH greater than 5,5, amorphous urates will be the major crystal form. Below 5.5, uric acid crystals are observed. It is not rare to observe calcium oxalate crystals with amorphous urate in the same urinary sediment. Urate crystals seem to have an enhancer effect on the calcium oxalate crystal formation. A possible explanation is that urates and oxalates are competing for the litho-inhibitor macromolecules. Hypocitraturia The chelating effect of citrate is known to reduce the saturation in calcium salt. Also, the soluble chelating calcium complex seems to have an inhibiting effect on crystal formation. One can therefore expect an increase in crystals formation, and even urolithiasis in conditions leading to hypocitraturia. Hypocitraturia is seen in conditions like in: • renal tubular acidosis, especially of the distal type. (RTA type I) • chronic diarrhea. • excessive animal protein intakes. Many bacteria infecting the urinary tract reduce the citrate concentration. 5% of the hypocitraturia are of unknown causes. Uric acid Approximately 66 to 75% of the uric acid is eliminated by the urine. The quantity to eliminate depends mostly on the diet (meat). In increased uricosuria, values > 4,5 mmol/d are observed. Uric acid crystals are formed when the urinary pH is <5,5 since the pK of uric acid is 5,5. Uric acid crystalluria is mainly due to a poor dilution volume at an acid pH, or due to an overproduction. In the majority of cases, this finding is of little clinical value and represents a pinpoint situation. Acid pH Some conditions, like chronic diarrhea, can be responsible for uric acid urolithiasis. Many of these patients will also have calcium stones. Overproduction Uric acid stones are seen in cases of gout, myeloproliferative syndrome, glycogenosis and neoplasms. Kidney Stones Symptoms and Signs Kidney stones (renal lithiasis) are small, hard deposits of mineral and acid salts on the inner surfaces of kidneys. Normally, the substances that make up kidney stones are diluted in the urine. When urine is concentrated, though, minerals may crystallize, stick together and solidify. The result is a kidney stone. Most kidney stones contain calcium. When a tubular structure is blocked in the body, pain is generated in waves as the body tries to unblock the obstruction. These waves of pain are called colic. This is opposed to noncolicky type pain, like appendicitis or pancreatitis, in which movement causes increased pain and affected people hold very still. Renal colic (renal is the medical term for things related to the kidney) has a classic presentation when a kidney stone is being passed. The pain is intense and comes on suddenly. It may wax and wane, but there is usually a significant underlying ache between the acute spasms of pain. 6 It is usually located in the flank or the side of the mid back and radiates to the groin. Those affected cannot find a comfortable position, and many writhe in pain. • Pain in the side and back, below the ribs • Fluctuations in pain intensity, with periods of pain lasting 20 to 60 minutes • Pain waves radiating from the side and back to the lower abdomen and groin • Bloody, cloudy or foul-smelling urine • Pain on urination • Sweating, nausea and vomiting • Persistent urge to urinate • Fever and chills if an infection is present

Blood may be visible in the urine because the stone has irritated the ureter. Blood in the urine (hematuria), however, does not always mean a person has a kidney stone. There may be other reasons for the blood, including kidney and bladder infections, trauma, or tumors. Urinalysis with a microscope may detect blood even if it is not appreciated by the naked eye. Sometimes, if the stone causes complete obstruction, no blood may be found in the urine because it cannot get past the stone. Exams and Tests The classic presentation of renal colic associated with blood in the urine suggests the diagnosis of kidney stone. Many other conditions can mimic this disease, and the physician or health-care provider may need to order tests to confirm the diagnosis. One example is that pain radiating to the back, may cause the health-care provider to be concerned about the possibility of a leaking abdominal aortic aneurysm. Physical examination is often not helpful in patients with kidney stones, aside from the finding of flank (side of the body between the ribs and hips) tenderness. The examination is often directed to ensuring that other potentially dangerous conditions are not present. As examples, when examining the abdomen, the physician may look for a palpable mass that pulsates, which may be a sign of an aortic aneurysm. Tenderness under the right rib cage margin may signal gallbladder disease. Tenderness in the lower quadrants may be associated with appendicitis, diverticulitis, or ovarian disease. Symptom control is very important, and medication for pain and nausea may be provided before the confirmation of the diagnosis occurs. A urinalysis may detect blood in the urine. It is also done to look for evidence of infection, a complication of kidney stone disease. Blood tests are usually not indicated, unless the health-care provider has concerns about the diagnosis or is worried about kidney stone complications. Computerized tomography (CT) scanning of the abdomen without oral or intravenous contrast dye is the most commonly used diagnostic test. The scan will demonstrate the anatomy of the kidneys, ureter, and bladder and can detect a stone, its location, its size, and whether it is causing dilation of the ureter and inflammation of the kidney. The CT can also evaluate many other organs in the abdomen, including the appendix, gallbladder, liver, pancreas, aorta, and bowel. However, since no contrast material is used, there are some limitations to the detail that can be observed in the images of the scan. Ultrasound is another way of looking for kidney stones and obstruction and may be useful when the radiation risk of a CT scan is unwanted (for example, if a woman is pregnant). Ultrasound requires a specially trained person to obtain the images, and therefore, it may not always be available. In those patients who already have the diagnosis of a kidney stone, plain abdominal X-rays may be used to track its movement down the ureter toward the bladder.

Tests for individual control. 1. A child of four and a half years old presented with bad appetite, fatigue, vomiting and abdominal pains not associated with meals, the temperature of 37.5C. He had had acute

7 respiratory viral infection two weeks before when traces of protein in urine, leucocyturia (to 40 in the field of view) and microhematuria were recorded. Objective data: clean skin and mucous membranes. Soft and moderately tender abdomen. Not enlarged liver and spleen. Positive Pasternadsky’s symptom. What is the most probable diagnosis? A. Acute primary pyelonephritis. B. Acute appendicitis. C. Acetonemic syndrome. D. Biliary dyskinesia. E. Helminthic invasion.

2. An 8-month-old child presented with the temperature of 39.2o C, he was flaccid and pale, refused from taking meals, had single vomiting and frequent urination. Physical examination showed no abnormality. His urine was cloudy . What supplementary investigation can help to make the diagnosis? A. General urine analysis. B. General blood analysis. C. Blood analysis for sugar. D. Urine analysis for sugar from daily amount of urine. E. Coprocytogram.

3. A 10-year-old child had fever to 38.5C, worsening of health and appetite, appearance of periorbital shadows and moderate pain in the right loin. The hemogram showed: leucocytes – 14x10^9/l, stab neutrophils – 12%, segmental leucocytes – 72%., erythrocyte sedimentation rate – 35 mm/h. Urine analysis revealed yellow cloudy urine with residue fibrin flakes, leucocytes – 50-70 in the field of vision, active leucocytes and many bacteria. Bacteriuria – more than 10^5 in 1 ml of urine. Ultrasound examination of kidneys showed increased echogenicity of pelvic system on the right, interstitial edema. Make initial diagnosis. A. Acute pyelonephritis. B. Dystonia of right kidney. C. Acute cystitis. D. Dysmetabolic nephropathy. E. Dysplasia of right kidney.

4. An 8-year-old girl complains of aching pain in the loins for 4 days. She has increased body temperature to 38o C, frequent painful urination. The girl had acute respiratory viral infection a week ago. What investigation should be done first? A. General urine analysis. B. General blood analysis. C. Ultrasound examination. D. Plan urography. E. Zimnitskiy’s test.

5. An 8-month-old child has had acute respiratory viral infection. Physical and neurologic-and behavioral development correspond to her age. She complains of flaccidity, decrease of appetite, repeated vomiting, increase of body temperature during the last day to 38C, paleness, frequent urination of small portions. Tachycardia. Urine analysis showed: protein – 0.099 g/l, leucocytes – 15-20 in the field of view, bacteriuria - +++, mucus - +++. What disease can be supposed? A. Acute pyelonephritis. B. Dysmetabolic nephropathy. C. Acute glomerulonephritis. D. Phosphate-diabetes. E. Care deficiency.

8 6. A 9-year-old girl presented with increased body temperature -37.5 C, headache, flaccidity, weakness, decrease of appetite, abdominal pains, frequent and painfull urination. Acute pyelonephritis was suspected. Clinical urine analysis revealed: specific gravity – 1018, no protein, leucocytes – 10-15 in the field of view. What method of investigation will surely confirm the diagnosis of urinary system infection? A. Bacteriologic investigation of urine. B. Reberg’s test. C. Zimnitskiy’s test. D. General clinical blood analysis. E. Clinical blood analysis in dynamics.

7. A 13-year-old boy complains of pain in suprapubic region, frequent urination of small urine portions, fever - 37.7C. Urine analysis showed: proteinuria – 0.033g/l, fresh erythrocytes covering the field of vision, unessential amount of oxalate salts. What is the most probable diagnosis? A. Acute cystitis. B. Dysmetabolic nephropathy. C. Acute glomerulonephritis. D. Acute pyelonephritis. E. Urolithiasis.

8. In pyelonephritis there is no: A. Leucocytosis B. Leucocyturia of neutrophilic genesis C. Slight proteinuria. D. Leucocyturia of lymphocytic type. E. Microhematuria.

9. Pyelonephritis differs from cystitis by: A. Bacteriuria grade. B. Leucocyturia grade C. Presence of intoxication, great activity of inflammatory process. D. Cylindrouria grade. E. Presence of dysuric disorders.

10. Which of the signs listed below is not the criterion of lower urinary tract infection? A. Transient bacteriuria. B. Slight leucocyturia. C. Rapid normalization of urine analysis. D. Kidney dysfunction. E. Cylindrouria.

Tasks for individual control. 1. Girl of 9 years of age (28 kg) was hospitalized to the department with complications on pain in the back side, frequent urination. From the anamnesis was found that girl had passed rubella, chicken-pox, often ARVI. Allergological anamnesis without complications. Before the disease lowering of the temperature was signed. On the next day head ache, adynamia, abdominal pain and in the back side in the left appeared. Body temperatures increased to the 39 C. Catarrhal symptoms were not admitted. During the next 4 days fever continued, pollakiuria and urine became cloudy. At the moment of the hospitalization general condition of the girl is moderate. The skin is pale, swells are absent, body temperature is 38 C. Pasternatsky symptom is positive bilateral, more significantly in the left. Palpation of the left kidney is painful. More frequent urination is observed. General blood test: Hb – 120 g/l, leucocytes – 10,5x109/l, s – 10%,

9 seg – 60%, l – 22%, m – 8%, ESR – 25 mm/h. General urine test: reaction – slightly acidic, protein – 0,099 g/l, leucocytes – cover all r/v, erythrocytes – 1 in r/v, bacteria – lot. Biochemical blood test: urea – 4,3 mmol/l, clearance of creatinine – 0,05 mmol/l. 1. Please, put diagnose, explain it. 2. Create plan of examination. 3. Check renal function.

2. Boy of 12,5 years of age (25 kg) was hospitalized to the department with complications on pain in the back side, rare urination, headache. The child is from the II pregnancy that coursed with nephropathy in the third trimester. Before the 1 year of age had atopical dermatitis. Had mumps. The boy is ill since the birth: in urine tests - leucocyturia (to 10 in r/v), increasing of body temperature to 38,8 C. The first time was examined at age of 2 years, bilateral cysticurethral reflux of 4 – 5 stage was diagnosed, megaurether. Had passed antireflux operation. All this time was under urologist observance. During the examination: general condition is moderate, the skin is pale, dry. Swells are absent. Breathing is normal. Cordial tones are thick. BP – 130/90 mm hg.rt. Abdomen is mild. Pasternatsky symptom is positive bilateral. General blood test: Hb – 95 g/l, leucocytes – 11,0x109/l, s – 7%, seg – 71%, l – 17%, m – 5%, ESR – 26 mm/h. General urine test: protein – 0,3 g/l, leucocytes – cover all r/v, erythrocytes – 0 - 1 in r/v, bacteria – lot. Biochemical blood test: general protein – 66 g/l, urea – 12,4 mmol/l, cholesterol – 4,4 mmol/l. 1. Please, put diagnose, explain it. 2. Create plan of examination. 3. Check renal function.

3. Boy of 10 years of age (30 kg) was hospitalized to the department with complications on abdominal pain and during urination, lowering of the appetite, increasing of the body temperature to 38,2 C. Family anamnesis: mother is healthy; father has hypertension and kidney disease that manifests with haematuria, proteinuria, and oxalate-calcium crystaluria. Grandmother from the father’s line also has kidney disease. The patient became ill 3 years ago, when after ARVI with continuous fever was found oxalate nephropathy. Treatment cause of this reason was not provided, diet was not followed. In week before the hospitalization suddenly appeared pain in back side, body temperature increased to the 38,5C, also bleeding was present. General condition of the patient is moderate. Abdomen is mild during palpation, ache long the urethras. Pasternatsky symptom is positive bilateral. General blood test: Hb – 110 g/l, leucocytes – 12,8x109/l, ESR – 18 mm/h. General urine test: protein – 0,099 g/l, leucocytes –20 – 25 in r/v, weight – 1030. Bacterial urine test: Escherichia coli – 200000/ml. Biochemical urine test: oxalates – 27 mmol/day (N – 9 – 13,5), calcium – 10 mmol/day (N – 1,5 - 4). 1. Please, put diagnose, explain it. 2. Create plan of examination. 3. Check renal function.

4. Girl of 8 years of age (25 kg) was hospitalized to the department with complications on pain in the back side and in abdomen, frequent painful urination. From the anamnesis was found that girl had ARVI 5 – 6 times a year. In 4 days before hospitalization was increasing of the body temperature up to 38,5 C, one time bleeding, abdominal pain. Was examined by children’s surgery – pathology was not found. At the moment of the hospitalization general condition of the girl is severe, fever, the skin is pale, red in chins, mucous membranes are dry. Breathing is normal. Cordial tones are rhythmical, loud. BP – 105/65 mm hg.rt. Abdomen is mild, painful during kidney palpation. Pasternatsky symptom is positive bilateral. More frequent and painful urination is observed. General blood test: Hb – 118 g/l, leucocytes – 10,5x109/l, s – 8%, seg – 70%, l – 17%, m – 5%, ESR – 24 mm/h. General urine test: protein – 0,033 g/l, leucocytes –20 – 25 in r/v, weight – 1010, bacteria – lot. Bacterial urine test: Escherichia coli – 120000/ml. Zymnitskiy test: DD – 250 ml, ND – 750 ml, urine weight – 1010 – 1020. Biochemical blood test:

10 general protein – 72 g/l, urea – 6,32 mmol/l, cholesterol – 4,8 mmol/l. Excretory urography: CPS is deformed. Urethras are spread. 1. Please, put diagnose, explain it. 2. Create plan of examination. 3. Check renal function.

5. Girl of 5 years of age acutely became ill: body temperature increased to 39 C, abdominal pain, frequent urination, the girl is crying during them. From the anamnesis is known that girl had intestinal dysbiosis, frequent ARVI. The girl attends kindergarten. One week before had ARVI. During the examination: general condition is moderate, the skin is pale. Catarrhal symptoms are not admitted. Breathing is normal. Cordial tones are rhythmical, loud. Abdomen is mild, painful during palpation. Liver + 0,5 cm. Urination is frequent. Defecation is normal. General blood test: Hb – 105 g/l, leucocytes – 10,5x109/l, s – 8%, seg – 60%, l – 24%, m – 8%, ESR – 35 mm/h. General urine test: protein – 0, 33 g/l, leucocytes –20 – 25 in r/v, weight – 1010, erythrocytes – fresh 4 – 5 in r/v, bacteria – lot. Biochemical blood test: general protein – 72 g/l, urea – 5,44 mmol/l. USS of kidneys. Kidneys are enlarged: right – 98x39 mm, left 92x35 mm. Echogenic moderately increased. Pelvis in the right side is spread to 9 mm, in the left side – normal up to 5 mm. Swells on the walls of the pelvic system. 1. Please, put diagnose, explain it. 2. Create plan of examination. 3. Check renal function.

6. Girl is 10 months of age (10 kg). Mother complains on increasing of body temperature up to 38,5 C, exciting, bleeding 2 times, lowering of the appetite. From the anamnesis is known that since the age of 3 month child is on the artificial feeding. At the age of 6 month received treatment concerning intestinal dysbiosis, one week before had ARVI. At the moment of the hospitalization general condition of the girl is severe, fever, excitement. Catarrhal symptoms are not admitted. The skin is clean, pale. Mucous membranes are clean, pale. Breathing is normal. Cordial tones are rhythmical, loud. Abdomen is mild, enlarged. Liver +2 cm, spleen – normal. Defecation – 3 times, without pathological elements. General blood test: Hb – 100 g/l, leucocytes – 10,8x109/l, s – 5%, seg – 65%, l – 22%, m – 8%, ESR – 27 mm/h. General urine test: protein – 0, 12 g/l, leucocytes –25 – 30 in r/v, weight – 1010, bacteria – lot. Biochemical blood test: general protein – 65 g/l, urea – 6,14 mmol/l. Nechiporenko test: leucocytes – 15000/ml, erythrocytes – 500/ml. USS of kidneys. Kidneys are enlarged, echogenic moderately increased. Swells on the walls of the pelvic system, not deformed. 1. Please, put diagnose, explain it. 2.Create plan of examination. 3. Check renal function.

7. Girl of 6 years of age (21 kg) bacame ill 3 days ago. Complications on frequent painful urination, vomiting, bleeding, increasing of the body temperature to 38,1 C, abdominal pain. During the examination the skin is pale. Mucous membranes are clean, pale. Cordial tones and breathing are normal. BP – 100/50 mm hg.rt. Pulse rate – 100/min. Abdomin during pulpation is mild, painful. Pasternatsky symptom is positive bilateral. Urination is painful, 15 times a day. General blood test: Hb – 110 g/l, leucocytes – 9,0x109/l, s – 5%, seg – 67%, l – 20%, m – 8%, ESR – 24 mm/h. General urine test: protein – 0, 183 g/l, leucocytes –35 – 40 in r/v, weight – 1012, bacteria – few, erythrocytes – 1 – 2 in r/v. Bacterial urine test: Escherichia coli – 120000/ml. Zymnitskiy test: General D – 1000 ml, DD – 450 ml, ND – 550 ml, urine weight – 1005 – 1015. USS of kidneys. Kidneys are not enlarged, pelvic system not deformed. Volume of cystic – 180 ml, after urination – 10 ml (rest urine). Pelvic before urination: left – 11 mm, right – 10 mm, after – from the both sides – 13 mm. 1. Please, put diagnose, explain it. 2. Create plan of examination.

11 3. Check renal function.

8. Boy is 2 years of age (12 kg). became ill acutely: body temperature increased to 38,6 C, appetite was lowered, one time bleeding observed, swells appeared. According to the mothers comments it was not the first time attack, the same situations appeared few times before. In urine test leucocyturia was found, but explain all changes like ARVI and phymosis. During the examination general condition is severe: increased temperature. Catarrhal symptoms are not admitted. The skin is clean, pale. Mucous membranes are clean, pale. Breathing is normal. Cordial tones are rhythmical, loud. Abdomen is mild, painful during palpation. Urination is frequent. Defecation is normal. General blood test: Hb – 100 g/l, leucocytes – 10,5x109/l, s – 7%, seg – 67%, l – 20%, m – 6%, ESR – 32 mm/h. General urine test: protein – 0, 24 g/l, leucocytes –25 – 30 in r/v, weight – 1005, bacteria – lot, erythrocytes – 1 – 2 in r/v. Nechiporenko test: leucocytes – 9500/ml, erythrocytes – 1000/ml. Biochemical blood test: general protein – 62 g/l, urea – 5,12 mmol/l, clearance of creatinine – 0,05 mmol/l, calcium – 2,2 mmol/l. USS of kidneys: right kidney is not enlarged, deformed due to uncompleted duplicate; left kidney – smaller – 50x25 mm (hypoplasia). 1. Please, put diagnose, explain it. 2. Create plan of examination. 3. Check renal function.

9. Girl of 12 years of age (25 kg) was hospitalized to the department with complications on pain in the down part of abdomen, increasing of body temperature up to 38,5 C. Became ill acutely, before the disease lowering of the temperature was signed. Previously the same symptoms were not admitted. During the examination: general condition is moderate, the skin is clean, pale. Girl has fever, headache. Swells are absent. Breathing is normal. Cordial tones are rhythmical, loud. BP – 100/50 mm hg.rt. Abdomen is mild, painful during palpation. Renal palpation painless. Pasternatsky symptom is negative bilateral. Urination is painful, 15 times a day. General blood test: Hb – 120 g/l, leucocytes – 7,2x109/l, s – 2%, seg – 65%, l – 25%, m – 8%, ESR – 15 mm/h. General urine test: protein – 0, 033 g/l, leucocytes –5 – 6 in r/v, weight – 1020, bacteria – absent, erythrocytes – 8 – 10 in r/v. Nechiporenko test: leucocytes – 3000/ml, erythrocytes – 2000/ml. Zymnitskiy test: General D – 1400 ml, DD – 1000 ml, ND – 400 ml, urine weight – 1010 – 1025. Biochemical blood test: general protein – 68 g/l, urea – 4,5 mmol/l, clearance of creatinine – 0,05 mmol/l, calcium – 2,2 mmol/l, kalium – 4,5 mmol/l, natrium – 145 mmol/l. Roberg test: V – 750 ml/24 h, V1 – 0, 52 ml/min, C - 10, 2 mmol/l, F - 99, 8 ml/min, R – 99, 4% USS of kidneys. Kidneys are not enlarged. Swells on the walls of the pelvic system, not deformed. 1. Please, put diagnose, explain it. 2. Create plan of examination. 3. Check renal function.

10. Boy of 13 years of age (35 kg) was hospitalized to the department with complications on painful, frequent urination, abdominal pain and in the back side, increasing of body temperature up to 38,0 C. Before the disease lowering of the temperature was signed. Week ago boy had passed ARVI. The boy is ill for the second day. During the examination: general condition is moderate, the skin is clean, pale. Boy hasheadache. Swells are on the legs, face. Breathing is normal. Cordial tones are rhythmical, loud. Abdomen is mild, painless during palpation. Renal palpation painful. Pasternatsky symptom is positive bilateral. Urination is painful, frequent. General blood test: Hb – 110 g/l, leucocytes – 10x109/l, s – 7%, seg – 68%, l – 20%, m – 5%, ESR – 20 mm/h. General urine test: protein – 0, 33 g/l, leucocytes –25 – 35 in r/v, weight – 1022, bacteria –few, erythrocytes – 4 – 5 in r/v. Bacterial urine test: Escherichia coli – 200000/ml. Zymnitskiy test: General D – 1000 ml, DD – 450 ml, ND – 550 ml, urine weight – 1010 – 1024. Roberg test: V – 750 ml/24 h, V1 – 0, 52 ml/min, C - 10, 2 mmol/l, F - 99, 8 ml/min, R – 99, 4% USS of kidneys. Kidneys are not enlarged. Pelvic system is not deformed.

12 1. Please, put diagnose, explain it. 2. Create plan of examination. 3. Check renal function.

Recommended references Basic: 1. Nelson Textbook of Paediatrics, 2-Volume set, 21st Edition. Authors: Robert Kliegman Joseph St. Geme at al. - Elsevier Inc., 2019. – 4264р. (p.374-395). 2. Textbook of Paediatric Emergency Medicine, 3rd Edition. Editors: Peter Cameron, Gary J. Browne, Biswadev Mitra et al. - Elsevier, 2020. – 688р. 3. Practical Paediatric Prescribing, 1st Edition Will Carroll, Francis J Gilchrist, Michael Mitchell et al. - Elsevier, 2020. – 328р. 4. Feigin and Cherry's Textbook of Pediatric Infectious Diseases, 8th Edition 2-Vol.- Elsevier, 2019. – 3152 р. 5. Paediatric Clinical Examination Made Easy, 6th Edition Denis Gill, Niall O'Brien - Elsevier, 2018. – 312р. 6. Pediatrics: textbook / T.O.Kryuchko, O.Y.Abaturov, T.V.Kushnereva et al.: edited by T.O.Kryuchko,O.Y.Abaturov .-Kyiv:AUS Medicine Publishing, 2017.-208 p.

Additional 1. Pediatry. Guidance aid / Ed. by O. Tiazhka – Kyiv: Medicine, 2007. 2. Behrman Richard E., Robert M. Kliegman, Hal B. Jenson, eds. Nelson Texbook of Paediatrics. Philadelfia: WB Saunders, 18th edition - 2012. – 994 р. – Р.638. 3. Illustrated Textbook of Paediatrics/ Ed.: T. Lissauer, G. Clayden.- Edinburg: Mosby, 2006.- 410 pp. 4. Forfar & Arneil’s Textbook of Pediatrics/ Ed.: N.Mc Intosh, P.J. Helms, R.L. Smyth, S. Logan.- Edinburg: Churchill Livingstone, 2008. - 1687 pp.

Information resources http://onlinelibrary.wiley.com/doi/10.1002/14651858.CD008117.pub2/full http://pediatrics.aappublications.org http://www.generalpediatrics.com http://www.aap.org/en-us/Pages/Default.aspx http://hospitalpediatrics.org https://www.duodecim.fi/ The Finnish Medical Society Duodecim http://www.uptodate.com https://www.nice.org.uk/ National Institute for Health and Clinical Excellence (NICE) http://www.awmf.org The Association of the Scientific Medical Societies in Germany http://www.has-sante.fr/ The French National Authority for Health https://www.ama-assn.org/ AMA (American Medical Association) www.nice.org.uk www.medscape.com www.bmj.com The methodical instructions are compiled by Associate Prof Poda O.A.

Methodical instructions were discussed and approved at the meeting of department of Pediatrics №2 protocol № ___ of ______20____.

Additions and changes were made “____” ______20____ Additions and changes were made “____” ______20____ Additions and changes were made “____” ______20____ Additions and changes were made “____” ______20____ 13 Additions and changes were made “____” ______20____ MINISTRY OF HEALTH OF UKRAINE UKRAINIAN MEDICAL STOMATOLOGICAL ACADEMY

“APPROVED” at the meeting of department of Pediatric № 2 “_05”_06__2020 Protocol № 1 Head of the department MD, Prof. Kryuchko T.O. ______

Methodical instructions for independent work of students during preparation to practical classes and in class Academic discipline Pediatrics

Module # 6 Topic of the lesson Glomerulonephritis in children. Course 4 Faculty Medical faculty

Poltava 2020 1. Relevance of the topic: The diseases of buds are wide-spread among child's population, their frequency makes 29 on a 1000 population. Among the diseases of kidneys one of central places is occupied by different forms of glomerulonephritis, 4-12 10000 children fall ill in a year. 4-12 10000 children. Usually a streptococcus infection as pharyngitis, tonsillitis, scarlatina precedes to glomerulonephritis, skinning displays are impetigo. Assume that cooling, respirator viral infection at a child with chronic tonsillitis or transmitter of skinning nephritogenic culture of streptococcus A can result in activating of infection and stipulate the origin of glomerulonephritis. Thus, children have glomerulonephritis by an important medico-social problem, with the clinical side of which the doctor of any specialty must be acquainted. The evaluation of is challenging, despite many advances in diagnosis and treatment over the past decade. CRF is the result of slowly progressive kidney diseases and seldom is fully reversible. This condition in childhood is associated with obstructive uropathy, congenital aplastic/hypoplastic/dysplastic kidneys, and other causes. In CRF, almost every system in the body eventually becomes compromised. 2. The specific aims: − to estimate etiological and pathogenesis factors of glomerulonephritis in children; − to estimate main etiopathogenic reasons that influence development of acute and chronicle forms of glomerulonephritis; − to classify and analyze typical clinical picture of glomerulonephritis in children; − to create plan of examination and analyze results of laboratory and instrumental investigations in case of glomerulonephritis in children: general clinical and biochemical blood tests, general urinary test, methods of quantity estimation of blood components and proteinuria, echographia, nephrobiopunction; − to provide differential diagnostic and put previous diagnose in case of glomerulonephritis in children; − to demonstrate skills of treatment, rehabilitation and prevention of glomerulonephritis in children; − to demonstrate skills of ethical and deontological principles of medical worker and principles of subordination in children’s nephrology

3. Basic knowledge, skills, habits necessary for studing of the topic (inter-disciplinary integration) The names of the preceding disciplines The acquired skills Normal anatomy, Physiology Anatomic and physiologic features of the urinary system in children. Pathologic physiology Pathophysiologic mechanisms of glomerulonephritis. Determine the etiology of glomerulonephritis based on knowledge of pathophysiologic mechanisms. Biochemistry To give a clinical assessment of changes in biochemical parameters of blood and urine Pharmacology and clinical pharmacology Determine the indications for therapy, calculate the dose of drugs, prescribe prescriptions. Propedeutics of pediatric diseases Physical examination of the urinary system in children. Perform physical examination of urinary system (gross inspection, palpation, percussion).

4. Tasks for independent work in preparation for class and in class. 4.1. List of the main terms and characteristics to know during the preparation to practice: Term Determination Group of heterogenic, cyclic coursing infectiously-allergic 1. Glomerulonephritis diseases of kidneys which are characterized, above all things, by the defeat of glomerular part Cyclic coursing infectiously-allergic disease, 2. Acute glomerulonephritis that is characterized at the typical course by the outbreak with visible by hematuria and edema Group of heterogeneous primary glomerulopathy, that is characterized by persistent making progress inflammatory, 3. Chronic sclerotic and destructive processes with the subsequent defeat glomerulonephritis and other departments ofnephron, in particular, by a tubulo- intersticial sclerosis

4.2. Theoretical questions to employment: 1. Etiology of acute and chronic glomerulonephritis. 2. Factors which due weight of motion of acute and chronic glomerulonephritis. 3. Clinical forms of acute and chronic glomerulonephritis in child's age. 4. Clinical description of course of acute glomerulonephritis. 5. Clinical description of course of chronic glomerulonephritis. 6. Diagnostics of acute and chronic glomerulonephritis. 7. Differential diagnostics of acute and chronic glomerulonephritis. 8. Treatment of children with acute glomerulonephritis . 9. Treatment of children with chronic glomerulonephritis. 11. Prophylaxis of acute and chronic glomerulonephritis

4.3. Practical tasks which are executed on employment: 1. Work with the test tasks. 2. Work of students near the bed of children patient with acute and chronic glomerulonephritis. 3. To diagnose the disease: acute and chronic glomerulonephritis. 4. To expose the states which threaten to life of child. 5. To work out a plan of inspection of children patient with acute and chronic glomerulonephritis. 6. To appoint the proper treatment and give urgent help in case of occurring of complications. 7. Clinical analysis of model case. 8. Decision of situation tasks.

Content of the topic. Glomerulonephritis - is a primary or secondary immune-mediated disease characterized by inflammation of the glomeruli. The classification − acute, sub-acute and chronic. − The glomerulonephritis is considered acute, if in 12 months all clinical and laboratory signs of pathological character disappear. A sign of convalescence is the complete remission during 5 years. − For acute glomerulonephritis the cyclic course is characteristic: (a) Initial period. (b) Intense period of the disease (2-3 weeks). c) Period of return development (2-3 months - 1 year). If during 1-1.5 years on the background of treatment and constant hospital observation of the child any clinical or laboratory symptoms of glomerulonephritis are still 3 observed, it is necessary to conceive about changing the disease transfer from the acute form to chronic. Nephrotic Syndrome: − Proteinuria in excess of 3.0 grams in 24 h − Edema − Hypoproteinemia, Hypoalbuminemia − Hyperlipidemia and lipiduria. Nephritic syndrome: − Haematuria − Variable proteinuria − Hypertension − Azotemia

Isolated urinary syndrome − Haematuria − Variable proteinuria − Hyaline and/or cellular casts

Nephrotic syndrome with Hematuria and Hypertension (Nephrotic syndrome + Nephritic syndrome)

Pathogenesis/ Antibody mediated glomerular injury usually occurs by one of four basic mechanisms. Antibodies may interact with: 1. Intrinsic components of the glomerular basement membrane. 2. Antigens on glomerular cells. 3. Exogenous antigens planted within the glomerulus. 4. Glomerular deposition of circulating immune complexes The filtration of albumin, a negatively charged molecule, is restricted to a greater extent than is the filtration of neutral dextran of an equivalent size. Molecular charge in addition to size influences the filtration of macromolecules across the glomerular capillary wall. Charge selectivity derives from electrostatic interactions (between fixed negatively charged components of the capillary wall and the macromolecule. Filtration of polycations is enhanced. Endothelial cells and the lamina rara intema provide the primary functional barrier to circulating polyanions. Most glomerulonephritis are antibody mediated and include: 1. - Postinfectious glomerulonephritis. 2. Membranous glomerulonephritis. 3. - IgA nephropathy. 4. - Membranoproliferative glomerulonephritis. 5. - Antiglomerular basement membrane disease.

Post-Infectious Glomerulonephritis. The precise nature of the antigens involved in the formation of the nephritogenic immune complexes is unknown. Streptococcal antigenic substances have been inconsistently detected in glomeruli and circulating immune complexes have been detected in some patients. Since streptococcal antigens do not always cause disease, other mechanisms may be involved, including alterations in IgG or glomerular components making them immunogenic. Antigens derived from infectious agents may bind to glomerular structures and induce development of in situ immune complexes. Membranous Glomerulonephritis. Approximately 25 to 30% of cases are secondary. Common associations include: 1. Systemic lupus erythematous and other connective tissue disorders. 2. Drugs (gold, penicillamine, non-steroidal antiinflammatory agents). 4 3. Hepatitis B, syphilis, malaria, leprosy, schistosomiasis. 4. Carcinoma, melanoma, leukemia, non-Hodgkin’s lymphomas. IgA Nephropathy - first described in 1968, is the most common from of primary glomerulonephritis in the world. It is an antibody-mediated glomerular disease in which the immune deposits localize to the mesangium. It is not certain whether the deposit´s forms exist in situ or are connected with circulating immune complexes.

Focal segmental glomerulosclerosis may be primary (idiopathic) or secondary to a number of etiologic agents including: - Unilateral renal agenesis. - Renal ablation. - Sickle cell disease. - Morbid obesity (with or without sleep apnea) - Congenital cyanotic heart disease. - Heroin nephropathy. - HIV nephropathy - Aging kidney. Standards of paraclinic tests Laboratory tests A. Obligatory: the general blood test with determination of trombocytes the biochemical blood test with proteinogram, by determination of level of cholesterol, creatinin, urea general urine test evaluation of daily excretion of albumen analysis of urine on Nechyporenko, Zimnitskiy test immunological researches of blood with the ASL-O determination, IgG, M, And,, complement (С3 fraction), circulatory immune complexes (on possibility). B. Additional: Bloods: determination of antistreptocinase, determination of antibodies to the basal membrane of glomeruli and cytoplasm of neutrophils acid-base equilibrium determination of level of alkaline phosphatase, amilase lipidogramm determination of products of degradation of connecting tissue (CRP), albuminous-sedimentary tests (timol, Veltman’s), revmatoid factor determination of products of degradation of fibrin, protaminsulfate and etanol test virology researches for the evaluation markers of hepatitis inspection on a TORCH-infection determination of antibodies in composition Ig G, M to the viruses of measles, cytomegaly, herpes, antinuclear antibodies evaluation, LE-cages HLA-typical determination of interferon activity, determination of level of lisocyme determination of level is a beta-2-microglobuline research of transport of salts Urines: determination of osmolality research of transport of salts determination of level is a beta-2-microglobuline determination of level of lisocyme

Tests for individual control. 1. Mother of girl 7th years present complaints about recurrent stomach-aches and skin rash, promoted hyperhidrosis, diminishment of amount of urine and saturated its character. Nykturia is marked. BP - 90/60 mm Hg. General urine test: relative closeness of urine - 1028, albumen - 0,04 g/l, leucocytes - 9-10 in v.f., red corpuscles are changed 6-8 in v.f., cylinders - not observed, salts are oxalates generous amount. Set a preliminary diagnosis: 1. Acute glomerulonephritis with a nephritich syndrome 2. Infection of urinary tract 3. Tubulopathy 4. Dysmetabolic nephropathy 5. Acute kidney insufficiency

5 2. A child 9 years on the extent of 4 years treats oneself on an occasion chronic glomerulonephritis. It is on a clinical account. What inspection must be conducted for confirmation of violation of function of glomerules? 1. Dayly proteinuria 2. Clearance of endogenous kreatinine 3. The Zymnytskiy Test 4. General urine test 5. Level of rest nitrogen and urea in a blood

3. At a boy 3th years during the last week edema on face and on lower extremities. In lungs in lower parts dulling, the weakness of breathing. Edema of front abdominal wall. Ascites. BP - 90/50 mm Hg. the general urine test - albumen 4,2 g/l, leucocytes - 5-6 in v.f., red corpuscles - 2-3 in v.f., cylinders - hyaline 2-3 in v.f., albumen of blood - 48,6 g/l. Cholesterol of blood - 8,2 mM/l. A preliminary diagnosis is acute glomerulonephritis. Specify the clinical variant of disease:

1. Nephrotic syndrome 2. Nephritic syndrome 3. Nephrotic syndrome with hematuria 4. Nephrotic syndrome with hematuria and hypertension 5. Urinary syndrome

4. At a child 12 years, with acute glomerulonephritis with hypertension syndrome was exposed in the first days of disease. What value in pathogenesis of disease has angiotenzin II?

1. Brakes depression action of prostaglandines. 2. Promotes the products of erythropoietins. 3. Strengthens products and secretion of aldosterone. 4. Multiplies the cardiac troop landing. 5. Promotes the level of renine.

5. At a child 7 years in 10 days after the carried quinsy urine acquired the type of "meat pigwashes", head pain, weakness, edema of eyelids are appeared. BP - 130/90 mm Hg. It is selected 500 ml urines for days. In GBT: leucocytes 12х109/л, eoz. - 2 %, stab – 12 %, segment. 68 %, lymphocytes 19%, monocytes – 4 %, ERS - 28 mm/h. GUT : red corpuscles are all field of vision, albumen - 0,98 g/l, red corpuscles, cylinders. Ultrasound of kidneys: edema of parenchyma of both kidneys, layers of parenchyma not differentiated. Domestic anamnesis is not burdened. What variant of glomerulonephritis has a child ? 1. Acute glomerulonephritis with a nephrotic syndrome. 2. Acute glomerulonephritis with the isolated urinary syndrome 3. Acute glomerulonephritis with a nephritic syndrome 4. Acute glomerulonephritis with a nephrotic syndrome, hematuria and hypertension. 5. Chronic glomerulonephritis.

6. A boy 6 years was admitted with complaints about the face edema, headache, red color of urine. There is the BP 140/90 mm Hg at examination. In the analyses of urine of protein 1,2 g/l, red corpuscles are all field of vision. What diagnosis is most probable? 1. Tuberculosis of kidneys 2. Interstitial nephritis 3. Pyelonephritis 4. Acute glomerulonephritis 5. Nephrolithiasis

7. A boy 3 years was admitted with the expressed edema syndrome. Objectively: pale. BP - 90/60 mm Hg. Oliguria. The general urine test : a color yellow, relative density - 1020, albumen - 3,5 g/l, 6 Erythrocytes are leached, 4-5 in f.v., Leyk. - 2-3 in f.v. Dayly proteinuria is 6,6 g/l. Cholesterol - 9,8 mM/l. Acute glomerulonephritis with a nephrotic syndrome is supposed. What inspection must be conducted for confirmation of nephrotic variant of glomerulonephritis? 1. Rest nitrogen, urea of blood 2. Electrolytes of plasma 3. Zymnytskiy test 4. Proteinogramma 5. Nechyporenko urine test

8. At a child acute glomerulonephritis with a nephritic syndrome developed 6 years after the quinsy. What drugs is contra-indicated in therapy of patient? 1. Ampicillin 2. Erytromycin 3. Gentamycin 4. Cefazolin 5. Benzylpenicyllin

9. At a boy 12 years in 2 weeks head pain, nausea, weakness, appeared after ARVI, colors of urine "meat". A skin is pale, edema on face. BP 140/90 mm Hg, daily diuresis 400 ml. General blood test: red corpuscles 3,3#1012/l, Hb 100 g/l, leucocytes 17*109/l, eosinophil 2%, stab 10%, segment 65%, lymphocyte of 20%, monocytes 3%, ERS 34 mm/h. General urine test: albumen 0,6 g/l, red corpuscles on all field of vision, leucocytes 30-35 in f.v., cylinders are grainy. General albumen 60 g/l, 2-globulins 18%. What disease does a patient have? 1. Acute glomerulonephritis with the isolated urinary syndrome 2. Acute glomerulonephritis with a nephritic syndrome 3. Acute glomerulonephritis with a nephrotic syndrome 4. Interstitial nephritis 5. Acute pyelonephritis

10. A child within 5 years is on a clinical account at a nephrologist with a diagnosis chronic glomerulonephritis, mixed form in the stage of clinical remission. What changes of urine are most characteristic in this case ? 1. Leykocyturyya. 2. Bakteriuria. 3. Glucuria. 4. Haematuria. 5. Cylindruria

Tasks for self-control: Task 1. Helen К, 4 years, became ill sharply after carried 3 weeks ago quinsies. The edema of person, trunk, extremities, ascites, appeared. BP – 100 mm рт.ст. An urines general is albumen of 6,6‰, specific gravity - 1026, leyk.-1- 3 in п/з. Eras -2-3 in п/з, cylinders -6-7 in п/з. Day's proteinuria 8 gr. Diuresis 250 ml. General albumen of the blood 52г/л. Cholesterol - 9,6 mmоl/l. Albumens-23%.

Task. 1. To ground a diagnosis. 2. To work out a plan of inspection of patient. 3. To transfer principles of treatment. 4. To write out a recipe on prednyzolon to this child.

Task 2. A boy 12 years with edemata on all body, decline of dysuria, stomach-ache. Became ill sharply after carried 2 weeks ago quinsies. Objectively: the state is heavy, pale, anasarca. An.

7 bloods general – Hb - 70г/л, Er. 3,5 х 1012/л, L- 9,6 х 109/л, ESR-50 mm/h. In urinalyses - albumen 3,5‰, leyk.-1-2 in п/з. Er.-20-40 in f.v., cylinders - 6-7 in f.v. Dayly proteinuria -4,8g/l. Zymnytskiy Test: diuresis - 350 ml, DD- 200,0ml, ND-150,0ml, density 1015-1020. General albumen of blood-56 g/l. R.Nitrogen – 21 mmol/L. Urea - 8,5 mmol/L, Kreatynin - 0,11 mmol/L, cholesterol – 15 mmol/L. Task. 1. To ground a diagnosis. 2. To work out a plan of inspection of patient. 3. To transfer principles of treatment. 4. To write out a recipe on azatyoprin. to this child.

Task 3. Boy 12 years, grumbles about head pain, weakness, reddish color of urine. It is ill during 2 weeks, became ill in 3 weeks after the carried quinsy. At examination pallor of skin and mucous, a language is assessed by the white raid. Pulse 88 for 1 minute. The Pasternatsky symptom is positive on both side. In the analysis of urine: specific gravity 1012, albumen 2,97 g/l., leucocytes 2-4 in f.v., Er 25-30 in f/v. Daily amount of urine 315 ml, nightly amount of urine 550 ml. Task. 1. To ground a diagnosis. 2. To work out a plan of inspection of patient. 3. To transfer principles of treatment. 4. To write out to pentoksyfyllyn this child.

Task 4. Boy 9 years, acted with complaints about a stomach-ache, increase of temperature to 38°С, expressed edemata on face, extremities, trunk, diminishment of amount of urine. In anamnesis are frequent quinsies. Three weeks ago was ill a respirator viral infection. At the inspection in the analysis of urine: specific gravity 1016, albumen 3,94 g/l., leucocytes 5-6 in f/v, Er 2-3 in f/v, cylinders gyal. 1-2 in f/v. In the biochemical blood test: albumen - 52,9 g/l., urea 5,8 mmol/L, kreatynyn 0,084 mmol/L, cholesterol 7,28 mmol/L.

Task. 1. To ground a diagnosis. 2. To work out a plan of inspection of patient. 3. To transfer principles of treatment. 4. To write out to furosemyd this child.

Task 5. A girl is 8 years. on stationary treatment - 1,5 months. Two months ago carried a quinsy, whereupon head pain, weakness, appeared. The edemata on face increased before the receipt, appeared on extremities trunk. At a review the edemata are absent, a skin is pale. CHSS 80 in a minute. BP - 110/70 mm Hg. Biochemical blood test: albumen 50 g/l., cholesterol 10 mmol/L, urea 6 mmol/L, Kreat. 0,088 mmol/L. Global analysis of urine: specific gravity 1010, albumen 5,8 g/l, leucocytes 4-5 in f/v, Er 0-1 in f/v, gyal. cylinders 1-2 in f/v. The Zymnytsky test: specific gravity 1006-1020, daily diuresis prevails. Task. 1. To ground a diagnosis. 2. To work out a plan of inspection of patient. 3. To transfer principles of treatment. 4. To write out to enalapryl this child.

Recommended references Basic: 1. Nelson Textbook of Paediatrics, 2-Volume set, 21st Edition. Authors: Robert Kliegman Joseph St. Geme at al. - Elsevier Inc., 2019. – 4264р. (p.374-395).

8 2. Textbook of Paediatric Emergency Medicine, 3rd Edition. Editors: Peter Cameron, Gary J. Browne, Biswadev Mitra et al. - Elsevier, 2020. – 688р. 3. Practical Paediatric Prescribing, 1st Edition Will Carroll, Francis J Gilchrist, Michael Mitchell et al. - Elsevier, 2020. – 328р. 4. Feigin and Cherry's Textbook of Pediatric Infectious Diseases, 8th Edition 2-Vol.- Elsevier, 2019. – 3152 р. 5. Paediatric Clinical Examination Made Easy, 6th Edition Denis Gill, Niall O'Brien - Elsevier, 2018. – 312р. 6. Pediatrics: textbook / T.O.Kryuchko, O.Y.Abaturov, T.V.Kushnereva et al.: edited by T.O.Kryuchko,O.Y.Abaturov .-Kyiv:AUS Medicine Publishing, 2017.-208 p.

Additional 1. Pediatry. Guidance aid / Ed. by O. Tiazhka – Kyiv: Medicine, 2007. 2. Behrman Richard E., Robert M. Kliegman, Hal B. Jenson, eds. Nelson Texbook of Paediatrics. Philadelfia: WB Saunders, 18th edition - 2012. – 994 р. – Р.638. 3. Illustrated Textbook of Paediatrics/ Ed.: T. Lissauer, G. Clayden.- Edinburg: Mosby, 2006.- 410 pp. 4. Forfar & Arneil’s Textbook of Pediatrics/ Ed.: N.Mc Intosh, P.J. Helms, R.L. Smyth, S. Logan.- Edinburg: Churchill Livingstone, 2008. - 1687 pp.

Information resources http://onlinelibrary.wiley.com/doi/10.1002/14651858.CD008117.pub2/full http://pediatrics.aappublications.org http://www.generalpediatrics.com http://www.aap.org/en-us/Pages/Default.aspx http://hospitalpediatrics.org https://www.duodecim.fi/ The Finnish Medical Society Duodecim http://www.uptodate.com https://www.nice.org.uk/ National Institute for Health and Clinical Excellence (NICE) http://www.awmf.org The Association of the Scientific Medical Societies in Germany http://www.has-sante.fr/ The French National Authority for Health https://www.ama-assn.org/ AMA (American Medical Association) www.nice.org.uk www.medscape.com

The methodical instructions are compiled by Associate Prof Poda O.A.

www.bmj.com Methodical instructions were discussed and approved at the meeting of department of Pediatrics №2 protocol № ___ of ______20____.

Additions and changes were made “____” ______20____ Additions and changes were made “____” ______20____ Additions and changes were made “____” ______20____ Additions and changes were made “____” ______20____ Additions and changes were made “____” ______20____

9 MINISTRY OF HEALTH OF UKRAINE UKRAINIAN MEDICAL STOMATOLOGICAL ACADEMY

“APPROVED” at the meeting of department of Pediatric № 2 “ 05 ”06 2020 Protocol № 21 Head of the department MD, Prof. Kryuchko T.O. ______

Methodical instructions for independent work of students during preparation to practical classes and in class

Academic discipline Pediatrics

Module # 6 Topic of the lesson Acute renal failure in children. Hemolytic-uremic syndrome. Chronic renal disease. Course 4 Faculty Medical

Poltava 2020 1. Relevance of the topic: The evaluation of kidney failure is challenging, despite many advances in diagnosis and treatment over the past decade. Acute renal failure (ARF) is characterized by the abrupt failure of the kidneys to regulate water and electrolyte homeostasis. ARFs in childhood due to hemolytic- uremic syndrome, postinfectious acute glomerulonephritis, or dehydration are reversible, but a small percentage may progress to chronic renal failure (CRF). CRF is the result of slowly progressive kidney diseases and seldom is fully reversible. This condition in childhood is associated with obstructive uropathy, congenital aplastic/hypoplastic/dysplastic kidneys, and other causes. In CRF, almost every system in the body eventually becomes compromised.

2. The specific aims: − to estimate etiological and pathogenesis factors of acute renal failure, hemolytic-uremic syndrome, chronic renal disease in children; − to estimate main etiopathogenic reasons that influence development of acute renal failure, hemolytic-uremic syndrome, chronic renal disease in children; − to classify and analyze typical clinical picture of the acute renal failure, hemolytic-uremic syndrome, chronic renal disease in children; − to create plan of examination and analyze results of laboratory and instrumental investigations in case of acute renal failure, hemolytic-uremic syndrome, chronic renal disease in children: general clinical and biochemical blood tests, general urinary test, methods of quantity estimation of blood components and proteinuria, estimation of bacteriuria, fractional cystographia and excretory urographia, echographia, radiological exploration, nephrobiopunction; − to provide differential diagnostic and put previous diagnose in case of acute renal failure, hemolytic-uremic syndrome, chronic renal disease in children; − to demonstrate skills of treatment, rehabilitation and prevention of acute renal failure, hemolytic-uremic syndrome, chronic renal disease in children in children; − to demonstrate skills of ethical and deontological principles of medical worker and principles of subordination in children’s nephrology 3. Basic knowledge, skills, habits necessary for studing of the topic (inter-disciplinary integration) The names of the preceding disciplines The acquired skills Normal anatomy, Physiology Anatomic and physiologic features of the urinary system in children. Pathologic physiology Pathophysiologic mechanisms of acute renal failure, hemolytic-uremic syndrome, chronic renal disease. Determine the etiology of acute renal failure, hemolytic-uremic syndrome, chronic renal disease based on knowledge of pathophysiologic mechanisms. Biochemistry To give a clinical assessment of changes in biochemical parameters of blood and urine Pharmacology and clinical pharmacology Determine the indications for therapy, calculate the dose of drugs, prescribe prescriptions. Propedeutics of pediatric diseases Physical examination of the urinary system in children. Perform physical examination of urinary system (gross inspection, palpation, percussion).

4. Tasks for independent work in preparation for class and in class. 4.1. List of the main terms and characteristics to know during the preparation to practice: Term Definitions Hemolytic uremic syndrome is characterized by a microangiopathic hemolytic anemia, thrombocytopenia and acute renal failure. Acute renal failure characterized by rapid and sudden decrease in glomerular filtration and is clinically evident decrease in diuresis and violation excretion features water-electrolyte and other exchange defeat almost all organs and systems. Chronic renal failure nonspecific syndrome that develops as a result of hereditary, congenital and acquired kidney diseases due to progressive loss of nephrons and stroma with a steady decrease in the ability of the kidneys perform homeostatic functions.

4.2. Theoretical questions to employment: 1. Etiology of acute and chronic renal failure, hemolytic uremic syndrome. 2. Factors that determine the severity of the course of acute and chronic renal failure, hemolytic uremic syndrome. 3. Recognize and delineate the causes of acute renal failure. 4. Formulate emergency management of fluid electrolyte disorders in acute renal failure. 5. Clinical characteristics of the course of hemolytic uremic syndrome. 6. Treatment of children with hemolytic uremic syndrome. 7. Characterize the incidence, causes, and costs of chronic renal failure in children. 8. Explain the pathogenesis and treatment of complications of chronic renal failure. 9. Delineate a plan to help the family with the outcome of renal failure.

4.3. Practical tasks which are executed on employment: 1. Work with the test tasks. 2. Work of students near the bed of children patient with acute and chronic renal failure, hemolytic uremic syndrome. 3. To diagnose the disease: acute and chronic glomerulonephritis. 4. To expose the states which threaten to life of child. 5. To work out a plan of inspection of children patient with acute and chronic glomerulonephritis. 6. To appoint the proper treatment and give urgent help in case of occurring of complications. 7. Clinical analysis of model case. 8. Decision of situation tasks.

Content of the topic. Acute renal failure (ARF) is defined as an acute decline in renal function characterized by an increase in blood urea nitrogen (BUN) and serum creatinine values, often accompanied by hyperkalemia, metabolic acidosis, and hypertension. Significant morbidity and mortality can accompany ARF. Patients who have ARF recover their renal function either partially or completely or they develop end-stage renal disease. They also may develop associated multiorgan disease. ARF is divided into three forms: prerenal failure (most common), intrinsic renal failure, and postrenal failure. Treatment ranges from conservative medical management to or renal transplantation, depending on the severity of kidney disease and degree of renal function recovery. Worldwide, most cases of ARF in children are due to hemolytic-uremic syndrome or volume depletion. In prerenal failure, clinical history should reveal causes of volume depletion, such as dehydration due to vomiting or gastroenteritis, hemorrhage, cardiac failure, or third-space fluid losses. Laboratory findings indicative of prerenal failure include decreased urine output, normal 3 urinary sediments, increased urine osmolality (>400.0 mOsm in the older child and >350.0 mOsm in the neonate), low urinary sodium (<10.0 mEq/L [10.0 mmol/L]), low fractional excretion of sodium (<1% in the older child and <2.5% in the newborn), and an increased BUN-to-creatinine ratio. Renal ultrasonography and renal scan findings should be normal. Renal or intrinsic renal failure describes parenchymal injury due to vascular spasm, intravascular coagulation, and microvascular injury. The most common causes of intrinsic renal failure include acute tubular necrosis, interstitial nephritis, hemolytic-uremic syndrome, glomerulonephritis, and nephrotoxic drugs. Postrenal failure results from obstruction to urinary flow. Causes of obstruction include renal calculi, bladder outlet obstruction, and internal or external ureteral compression. Clinical history may reveal dehydration, hypoxic-ischemic events, toxic ingestion, NSAID or other nephrotoxic medication use, signs and symptoms of sepsis, gross hematuria, or trauma. With intrinsic renal failure, the patient's decreased urine output can be described as oliguria (<0.5 mL/kg per hour in a child or <1 mL/kg per hour in an infant) or as anuria (no urine output). Laboratory examination of the urine sediment may demonstrate red blood cell casts, granular casts, and red blood cells, findings seen in glomerulonephritis. When evaluating for possible glomerulonephritis, other appropriate biochemical studies include streptococcal antibodies, hepatitis B and C panels, and complement studies. Medical management of ARF includes maintaining renal perfusion and fluid and electrolyte balance, controlling blood pressure, treating anemia, providing adequate nutrition, adjusting medications for the degree of renal impairment, and initiating renal replacement therapy (dialysis) when indicated. Hemolytic-uremic syndrome (HUS) is one of the most common etiologies for acute kidney injury and an important cause of acquired chronic kidney disease in children.1 HUS is generally classified into two main types: typical or diarrhea-associated (D+HUS) and atypical (aHUS) or diarrhea-negative HUS. HUS is defined as the combination of a microangiopathic hemolytic anemia with variable degrees of thrombocytopenia and renal failure. The syndrome usually occurs in previously healthy children and often is preceded by a gastrointestinal enteritis. Other systems may be involved, such as the central nervous system (CNS). When fever and CNS manifestations coexist, the distinction between HUS and thrombotic thrombocytopenic purpura (TTP) can be difficult. Chronic kidney disease (CKD) is characterized by an irreversible deterioration of renal function that gradually progresses to end-stage renal disease. The main aetiologic factors of CKD in children are represented by CAKUT, steroid-resistant nephrotic syndrome (SRNS), chronic glomerulonephritis (e.g. lupus nephritis, Alport syndrome) and renal ciliopathies.

Stage Description GFR (mL/min/1.73 m2) 1 Kidney damage with normal or increased GFR >90 2 Kidney damage with mild decrease in GFR 60–89 3 Moderate decrease in GFR 30–59 4 Severe decrease in GFR 15–29 5 Kidney failure <15 or dialysis As the kidney fails, major disturbances in calcium, phosphate, and acid-base metabolism develop, resulting in renal rickets and growth retardation. With the increasing recognition of the kidneys as an endocrine organ, CRF can be said to give rise to malfunctions of all endocrine systems. The lack of erythropoietin production associated with kidney failure results in anemia. Insulin resistance, thyroid, and other endocrine dysfunctions complicate CRF. Growth hormone resistance in uremia, coupled with renal osteodystrophy, contributes to the often. severe growth failure seen in children who have CRF. Anorexia frequently complicates the late stages, resulting in 4 nutritional deficiency. These and related complications of CRF are reviewed to provide the pediatrician with a better understanding of how to help the child who has this major organ failure and to address the family's concerns.

Differences in CKD Staging for Children 1. Staging of Chronic Kidney Disease does not include patients younger than 2 years old. 2. Duration of more than 3 months to define Chronic Kidney Disease does not apply to newborns and infants less than 3 months of age. 3. Urine protein can be used instead of urine albumin excretion in children. Outcomes guideline proposes the following definition of CKD: • Abnormalities of kidney structure or function that have been present >3 months, with implications for the health of the patient, and either of the following: • decreased glomerular filtration rate (GFR) Markers of kidney disease include any of the following: • proteinuria (urine dipstick ≥2+ or urine protein:creatinine ratio 5 × the upper limit of normal) • urine sediment abnormalities • electrolyte and other abnormalities caused by tubular disorders • histological abnormalities • structural abnormalities detected by imaging • history of renal transplantation.

Tests for individual control. 1.An 18-month-old boy is hospitalized after 2 days of diarrhea and persistent vomiting. He has had no wet diapers in the past 12 hours. As part of the evaluation, you obtain the following laboratory results: urine sodium, 25 mmol/L (25 mEq/L); urine creatinine, 60 mg/dL (5,304 mcmol/L); plasma creatinine, 0.8 mg/dL (70.7 mcmol/L). The diagnosis that best accounts for the clinical and laboratory findings is: A. Glomerulonephritis. B. Hemolytic-uremic syndrome. C. Polycystic kidney disease. D. Posterior urethral valves. E. Rotaviral infection.

2. An 18-month-old girl is hospitalized for diarrhea, persistent vomiting, and continued anuria overnight despite receiving 20 mL/kg normal saline as an intravenous bolus. The laboratory results this morning reveal a plasma creatinine of 1.0 mg/dL (88.4 mcmol/L), with a fractional excretion of sodium of 3%. The best choice of intervention at this time is to replace urinary output and: A. Infuse insensible water loss. B. Infuse insensible water loss adjusted for tissue catabolism. C. Infuse maintenance fluids. D. Maintain an open vein for medications only. E. Repeat a 20 mL/kg normal saline fluid bolus.

3. The most common cause of chronic renal failure in children requiring transplantation is: A. Familial nephritis. B. Hemolytic-uremic syndrome. C. Obstructive uropathy. D. Polycystic kidney disease. E. Reflux nephropathy.

5 4. A 7-year-old boy who has chronic renal failure due to posterior urethral valves has a plasma creatinine of 1.2 mg/dL (106 mcmol/L). At this level of renal function, optimal management would require: A. Calcium acetate. B. Captopril. C. Erythropoietin. D. No treatment. E. Transplantation.

5. The boy's renal function progressively deteriorates over the next several years. His growth can best be maximized in the years before transplantation by: A. Assuring intake of 120 kcal/kg per day. B. Injecting growth hormone weekly. C. Maintaining hemoglobin at 13 g/dL (130 g/L) or higher. D. Maintaining plasma bicarbonate at 16 mmol/L. E. Providing a protein-rich diet.

Tasks for self-control: Task 1. Boy, 1,5 years age suffered an acute enterocolitis. At the time of examination emptying returned to normal, but there were swelling and sharply decreased urine output. On examination are present massive edema of the face, trunk and extremities. The skin is dry and cold to the touch. Borders of the heart are in the normal range, tones muffled. Pulse - 64 in min., blood pressure - 100/70 mm Hg. Laboratory: er. -2,5 x 1012/L, Hb - 98 g/L, white blood cells - 6.2 x1012/L, ESR - 15 mm/h. Biochemical analysis of blood: protein - 44 g/L; electrolytes: K- 6.2 mmol/L, Ca - 2.0 mmol/L, Na - 135 mmol/L, blood creatinine - 124 mcmol/L, blood urea - 16.2 mmol/L. Glomerular filtration rate is 62 ml/min. Urine output is 280 ml. per day. Relative density of urine is 1038. Urine protein comprised in the presence of 5.2 g/L protein, many hyaline, granular cylinders and epithelial cells. 1. Make the diagnosis. 2. What additional methods of investigation would you suggest? 3. Prescribe the treatment: etiotropic, pathogenetic, symptomatic (drug, dose, course duration). 4. Possible complications of the disease.

Task 2. A 6-year-old boy is hospitalized because of edema of leg, abdomen. The mother notes the decrease of diuresis, disposable vomiting. A child was vaccinated two weeks ago. Physical examination reveals an anasarca, blood pressure is 90/55 mm Hg. In the analysis of blood: red blood cells - 4,8 x1012/l, Hb - 117 g/l, white blood cells -17.5 x 109/l, ESR - 46 mm/h, The serum creatinine is 157 mmol/L, blood urea nitrogen - 16 mmol/l, total protein - 32.2 g/l, albumin - 21 g/l, α1 -3.7%, α2-globulins - 34%: β- globulins - 4,9%, γ-globulіns - 26,0%; cholesterol - 11.5 mmol/l; HbsAg is negative. In the analysis of urine: protein - 6,2 g/l, red blood cells - 1-3 in a field of view, white blood cells - 6-8 in a field of view. Analysis of urine by Nechiporenko: white blood cells - 2000, red blood cells - 1500 in 1 ml. Analysis of urine by Zymnitsky: daily urine - 360 ml, specific gravity: 1006-1009; protein 1,47-5,86 g/l in 8 samples. Abdominal ultrasonography reveals a normal kidney`s size, parenchyma is markedly echogenic, ascytes, hydroperycardum. 1. Make the diagnosis. 2. What additional methods of investigation would you suggest? 3. Prescribe the treatment: pathogenetic, symptomatic (drug, dose, course duration).

6 Task 3. A 8-year-old female presents to the emergency department with a seizure. On initial evaluation, the patient appears to be post-ictal, but is otherwise okay. Initial vital signs are significant for blood pressure of 180/110. She has never had a seizure before. On further questioning, the patient`s parents report that she had several episodes of coca-cola colored urine a few days ago, and was seen by her doctor and given an antibiotic for presumed urinary tract infection. Over the last few days prior to her presentation, she has been complaining of headaches and ankle swelling. Otherwise the patient has been fine. She has never been hospitalized, takes no medications, and she has no known allergies. She is very seldom ill, and with the exception of a sore throat 2 weeks ago, she has had no other recent illnesses. In urine: the relative density - 1021, protein – 1,5 g/l, RBCs are leached and cover the whole vision field, hyaline cylinders - 4-6 in the field of vision. 4. Make the diagnosis. 5. What additional methods of investigation would you suggest? 6. Prescribe the treatment: pathogenetic, symptomatic (drug, dose, course duration).

Task 4. A 14 y.o. patient complains of nausea, vomiting, headache, shortness of breath. He had an acute nephritis being 10 y.o. Proteinuria was found out in urine. Objectively: a skin is greypale, the edema is not present. Accent of II tone above aorta. BP 140/100-180/100 mm Hg. Blood level of residual N2- 6,6 mmol/L, creatinine - 406 mmol/L. Day’s diuresis- 2300 ml, nocturia. Specific density of urine is 1009, albumin- 0,9 g/L, WBC- 0-2 in f/vis. RBC- single in f/vis., hyaline casts single in specimen. 1. Make the diagnosis. 2. What additional methods of investigation would you suggest? 3. Prescribe the treatment: pathogenetic, symptomatic (drug, dose, course duration).

Task 5. A 15-years-old patient has chronic glomerulonephritis. BP 165/110 mm Hg. Urine test reveals the following: the relative density is 1005, protein - 1,65 g/l, RBCs - 5-7 in the field of vision, WBCs - 2-3 in the field of vision. Blood creatinine - 0,350 mmol/l. Serum sodium - 148 mmol/l. 1. Make the diagnosis. 2. What additional methods of investigation would you suggest? 3. Prescribe the treatment: pathogenetic, symptomatic (drug, dose, course duration).

Task 6. The parents of a 3-year-old boy bring him to your office because he appears very pale and is less active than usual. He has had diarrhea for 2 days. The parents also note that his urine output has decreased substantially in the past 24 hours. Physical examination reveals marked pallor and diffuse abdominal tenderness. Laboratory results reveal a blood urea nitrogen level of 38.6 mmol/L, serum creatinine of 460 mcmol/L. The hemoglobin blood level is 60 g/L. In the urine: 25 to 50 white blood cells and too numerous to count red blood cells. 1. Make the diagnosis. 2. What additional methods of investigation would you suggest? 3. Prescribe the treatment: pathogenetic, symptomatic (drug, dose, course duration).

7 Recommended references Basic: 1. Nelson Textbook of Paediatrics, 2-Volume set, 21st Edition. Authors: Robert Kliegman Joseph St. Geme at al. - Elsevier Inc., 2019. – 4264р. (p.374-395). 2. Textbook of Paediatric Emergency Medicine, 3rd Edition. Editors: Peter Cameron, Gary J. Browne, Biswadev Mitra et al. - Elsevier, 2020. – 688р. 3. Practical Paediatric Prescribing, 1st Edition Will Carroll, Francis J Gilchrist, Michael Mitchell et al. - Elsevier, 2020. – 328р. 4. Feigin and Cherry's Textbook of Pediatric Infectious Diseases, 8th Edition 2-Vol.- Elsevier, 2019. – 3152 р. 5. Paediatric Clinical Examination Made Easy, 6th Edition Denis Gill, Niall O'Brien - Elsevier, 2018. – 312р. 6. Pediatrics: textbook / T.O.Kryuchko, O.Y.Abaturov, T.V.Kushnereva et al.: edited by T.O.Kryuchko,O.Y.Abaturov .-Kyiv:AUS Medicine Publishing, 2017.-208 p.

Additional 1. Pediatry. Guidance aid / Ed. by O. Tiazhka – Kyiv: Medicine, 2007. 2. Behrman Richard E., Robert M. Kliegman, Hal B. Jenson, eds. Nelson Texbook of Paediatrics. Philadelfia: WB Saunders, 18th edition - 2012. – 994 р. – Р.638. 3. Illustrated Textbook of Paediatrics/ Ed.: T. Lissauer, G. Clayden.- Edinburg: Mosby, 2006.- 410 pp. 4. Forfar & Arneil’s Textbook of Pediatrics/ Ed.: N.Mc Intosh, P.J. Helms, R.L. Smyth, S. Logan.- Edinburg: Churchill Livingstone, 2008. - 1687 pp.

Information resources http://onlinelibrary.wiley.com/doi/10.1002/14651858.CD008117.pub2/full http://pediatrics.aappublications.org http://www.generalpediatrics.com http://www.aap.org/en-us/Pages/Default.aspx http://hospitalpediatrics.org https://www.duodecim.fi/ The Finnish Medical Society Duodecim http://www.uptodate.com https://www.nice.org.uk/ National Institute for Health and Clinical Excellence (NICE) http://www.awmf.org The Association of the Scientific Medical Societies in Germany http://www.has-sante.fr/ The French National Authority for Health https://www.ama-assn.org/ AMA (American Medical Association) www.nice.org.uk www.medscape.com

The methodical instructions are compiled by Associate Prof Poda O.A.

www.bmj.com Methodical instructions were discussed and approved at the meeting of department of Pediatrics №2 protocol № ___ of ______20____.

Additions and changes were made “____” ______20____ Additions and changes were made “____” ______20____ Additions and changes were made “____” ______20____ Additions and changes were made “____” ______20____ Additions and changes were made “____” ______20____

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