Biliary Atresia Definition
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Nutritional Disturbances in Crohn's Disease ANTHONY D
Postgrad Med J: first published as 10.1136/pgmj.59.697.690 on 1 November 1983. Downloaded from Postgraduate Medical Journal (November 1983) 59, 690-697 Nutritional disturbances in Crohn's disease ANTHONY D. HARRIES RICHARD V. HEATLEY* M.A., M.R.C.P. M.D., M.R.C.P. Department of Gastroenterology, University Hospital of Wales, Cardiffand *Department ofMedicine, St James's University Hospital, Leeds LS9 7TF Summary deficiency in the same patient. The most important A wide range of nutritional disturbances may be causes of malnutrition are probably reduced food found in patients with Crohn's disease. As more intake, active inflammation and enteric loss of sophisticated tests become available to measure nutrients (Dawson, 1972). vitamin and trace element deficiencies, so these are being recognized as complications ofCrohn's disease. TABLE 1. Pathogenesis of malnutrition It is important to recognize nutritional deficiencies at an early stage and initiate appropriate treatment. Reduced food intake Anorexia Otherwise many patients, experiencing what can be a Fear of eating from abdominal pain chronic and debilitating illness, may suffer unneces- Active inflammation Mechanisms unknown Protected by copyright. sarily from the consequences of deprivation of vital Enteric loss of nutrients Exudation from intestinal mucosa nutrients. Interrupted entero-hepatic circulation Malabsorption Loss of absorptive surface from disease, resection or by-pass KEY WORDS: growth disturbance, Crohn's disease, anaemia, vitamin deficiency. Stagnant loop syndrome from strictures, fistulae or surgically created blind loops Introduction Miscellaneous Rapid gastrointestinal transit Effects of medical therapy Crohn's disease is a chronic inflammatory condi- Effects of parenteral nutrition tion ofunknown aetiology that may affect any part of without trace element supplements the gastrointestinal tract from mouth to anus. -
Does Your Patient Have Bile Acid Malabsorption?
NUTRITION ISSUES IN GASTROENTEROLOGY, SERIES #198 NUTRITION ISSUES IN GASTROENTEROLOGY, SERIES #198 Carol Rees Parrish, MS, RDN, Series Editor Does Your Patient Have Bile Acid Malabsorption? John K. DiBaise Bile acid malabsorption is a common but underrecognized cause of chronic watery diarrhea, resulting in an incorrect diagnosis in many patients and interfering and delaying proper treatment. In this review, the synthesis, enterohepatic circulation, and function of bile acids are briefly reviewed followed by a discussion of bile acid malabsorption. Diagnostic and treatment options are also provided. INTRODUCTION n 1967, diarrhea caused by bile acids was We will first describe bile acid synthesis and first recognized and described as cholerhetic enterohepatic circulation, followed by a discussion (‘promoting bile secretion by the liver’) of disorders causing bile acid malabsorption I 1 enteropathy. Despite more than 50 years since (BAM) including their diagnosis and treatment. the initial report, bile acid diarrhea remains an underrecognized and underappreciated cause of Bile Acid Synthesis chronic diarrhea. One report found that only 6% Bile acids are produced in the liver as end products of of British gastroenterologists investigate for bile cholesterol metabolism. Bile acid synthesis occurs acid malabsorption (BAM) as part of the first-line by two pathways: the classical (neutral) pathway testing in patients with chronic diarrhea, while 61% via microsomal cholesterol 7α-hydroxylase consider the diagnosis only in selected patients (CYP7A1), or the alternative (acidic) pathway via or not at all.2 As a consequence, many patients mitochondrial sterol 27-hydroxylase (CYP27A1). are diagnosed with other causes of diarrhea or The classical pathway, which is responsible for are considered to have irritable bowel syndrome 90-95% of bile acid synthesis in humans, begins (IBS) or functional diarrhea by exclusion, thereby with 7α-hydroxylation of cholesterol catalyzed interfering with and delaying proper treatment. -
Malabsorption and Exocrine Pancreatic Insuffiecienty (Pi)
MALABSORPTION AND EXOCRINE PANCREATIC INSUFFIECIENTY (PI) Pancreatic Insufficiency is a condition in which a person does not have enough enzymes and bicarbonate being delivered from the pancreas to the intestine for digestion. This causes mal- absorption of nutrients, failure to gain weight and grow, weight loss, vitamin and mineral deficiency, and gastrointestinal symptoms. Most people with CF have mal-absorption due to PI. Onset usually occurs in the first one to two years of life, often in early infancy, but can start at anytime. Symptoms of mal-absorption -Change in number of stools -Large, bulky stools -Stools may be bulky and soft -Greasy, oily or floating stools, oil in toilet water -Stools may smell worse than usual or normal -Rectal prolapse -Mal-absorption of calorie providing nutrients and poor weight gain or weight loss Fat …………………………………………….9 calories/gram Protein………………………………..…….4 calories/gram Complex Carbohydrate ……………..4 calories/gram -Results in poor weight gain, weight loss, poor growth, decreased immune function and decreased lung health. -Mal-absorption of FAT SOLUBLE VITAMIN and deficiency: Vitamin A, Vitamin D, Vitamin E, Vitamin K -Mineral deficiencies: Calcium, Zinc, Sodium, Chloride Learn more about vitamins and minerals at: http://www.cff.org/treatments/Therapies/Nutrition/Vitamins/ Tests to Diagnose PI and Mal-absorption -72 hr fecal fat test -Pancreatic Fecal Elastase Treatment of PI and Mal-absorption Pancreatic Enzyme Replacement Therapy (PERT) Pancreatic enzymes are taken with each meal, snack, breast feed, bottle , and drink that contains fat protein and or complex carbohydrate. Antacid and acid blocking medicines can be added to make enzymes work better Fat Soluble Vitamin Supplementation with special supplements made for mal-absorption are prescribed Each enzyme company offers programs that provide free nutritional support and/or CF therapy support High Calorie, high protein diet Even with PERT, not all calories and nutrients from food are absorbed as expected and calories and nutrients are lost and need replacement. -
Digestive Health Center Nutrition Services Nutrition Guidelines for Chronic Pancreatitis Patient Education
Digestive Health Center Nutrition Services Nutrition Guidelines for Chronic Pancreatitis Patient Education The pancreas is an organ that: Produces pancreatic enzymes to help digest (break down) food in the small intestine for absorption Makes hormones (such as insulin) to help control blood sugars Chronic pancreatitis is ongoing inflammation of the pancreas. Symptoms can be worse after eating. Symptoms include: Abdominal pain Nausea Vomiting Weight loss Fatty stools (stools may also float and/or have a foul odor) Malabsorption of nutrients can occur from poor digestion of food (due to reduced pancreatic enzyme activity), which will result in nutrients passing into the stools. This is seen especially with fat and fat soluble vitamins (A, D, E) as digestion of fat is highly dependent on pancreatic enzymes. In some cases, diabetes can develop if the pancreas is not able to make enough insulin to help control blood sugars, so blood sugars stay high. Nutritional Guidelines Follow a low fat diet, which for chronic pancreatitis is often restricted to 50 grams of fat, but could also range between 30-50 grams of fat depending on tolerance. If you have diabetes, eat recommended serving sizes of low fat carbohydrates to help control blood sugars (low fat/non fat dairy, fruits, vegetables, whole grains, beans, lentils etc). Information on serving sizes is available. Take pancreatic enzymes as prescribed by your doctor to treat malabsorption. Take the enzymes before each meal and snack. They will not work if taken at the end of the meal. 1 Low Fat Diet Tips Eat 4-6 small meals throughout the day Spread out your fat intake throughout the day Use butter, margarine and cooking oils sparingly Bake, grill, roast and/or steam foods. -
Guideline for the Evaluation of Cholestatic Jaundice
CLINICAL GUIDELINES Guideline for the Evaluation of Cholestatic Jaundice in Infants: Joint Recommendations of the North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition and the European Society for Pediatric Gastroenterology, Hepatology, and Nutrition ÃRima Fawaz, yUlrich Baumann, zUdeme Ekong, §Bjo¨rn Fischler, jjNedim Hadzic, ôCara L. Mack, #Vale´rie A. McLin, ÃÃJean P. Molleston, yyEzequiel Neimark, zzVicky L. Ng, and §§Saul J. Karpen ABSTRACT Cholestatic jaundice in infancy affects approximately 1 in every 2500 term PREAMBLE infants and is infrequently recognized by primary providers in the setting of holestatic jaundice in infancy is an uncommon but poten- physiologic jaundice. Cholestatic jaundice is always pathologic and indicates tially serious problem that indicates hepatobiliary dysfunc- hepatobiliary dysfunction. Early detection by the primary care physician and tion.C Early detection of cholestatic jaundice by the primary care timely referrals to the pediatric gastroenterologist/hepatologist are important physician and timely, accurate diagnosis by the pediatric gastro- contributors to optimal treatment and prognosis. The most common causes of enterologist are important for successful treatment and an optimal cholestatic jaundice in the first months of life are biliary atresia (25%–40%) prognosis. The Cholestasis Guideline Committee consisted of 11 followed by an expanding list of monogenic disorders (25%), along with many members of 2 professional societies: the North American Society unknown or multifactorial (eg, parenteral nutrition-related) causes, each of for Gastroenterology, Hepatology and Nutrition, and the European which may have time-sensitive and distinct treatment plans. Thus, these Society for Gastroenterology, Hepatology and Nutrition. This guidelines can have an essential role for the evaluation of neonatal cholestasis committee has responded to a need in pediatrics and developed to optimize care. -
Nutrition Management in the Adult Patient with Crohn's Disease
Review Article: Nutrition management in the adult patient with Crohn’s disease Nutrition management in the adult patient with Crohn’s disease Basson A, MS RD(USA)(SA) Lecturer and Hospital Student Internship Supervisor, University of the Western Cape Correspondence to: Abigail Basson, email: [email protected] Keywords: inflammatory bowel disease, nutrition therapy, Crohn’s disease Abstract Malnutrition, nutrient deficiencies and osteoporosis are common in patients with Crohn’s disease, regardless of disease activity. While the role of diet in the pathogenesis of the disease remains inconclusive, upon diagnosis, nutrition therapy plays an integral role in patient care. Successful nutrition intervention involves appropriate nutritional assessment, supplemental nutrition and individualised counselling and support. Peer reviewed. (Submitted: 2012-05-08. Accepted: 2012-11-04.) © SAJCN S Afr J Clin Nutr 2012;25(4):164-172 Introduction Low-fibre, high-fat and high-sugar intakes have been implicated as some of the environmental triggers in disease development, Crohn’s disease (CD) is a chronic and recurrent immune-mediated although the role of a pre-illness diet in the pathogenesis of CD inflammatory disorder of the gastrointestinal tract.1,2 Typically, pa- remains inconclusive.16-18 However, upon diagnosis, nutrition therapy tients suffer from chronic intestinal inflammation that follows a plays an integral role in patient care, regardless of disea se activity. relapse-remitting pattern, as well as from a variety of complications 3,4 (Table I) that may or may not involve the gut. Disease activity can Malnutrition be classified by the Crohn’s Disease Activity Index5 (CDAI) (Table II), and usually, treatment includes various combinations of corticoste- Weight loss, low body mass index (BMI) and nutrient deficiencies roid, anti-inflammatory (aminosalicylates), immune-modulating or have been well documented in patients with CD, especially during biological therapy.4 While the exact cause of CD is not known, it is active disease. -
Low Fructose Diet
Low Fructose Diet What is fructose? Fructose is a simple sugar found naturally in certain fruits, vegetables, and sweeteners. It is also added to some processed foods and is a component of regular table sugar (sucrose). What is fructose malabsorption? Some people cannot completely absorb fructose in the small intestine. When fructose is not absorbed it is quickly consumed by the bacteria that normally live in the gut. The result is abdominal bloating, cramping, gas, diarrhea, and/or constipation. Fructose malabsorption can occur in people with Irritable Bowel Syndrome (IBS) and other gastrointestinal (GI) disorders. It is diagnosed with a fructose hydrogen breath test and the treatment is a low fructose diet. What foods should I avoid? You do not have to avoid all foods that contain fructose, only those that are high in excess fructose listed below. Excess fructose means that the food has more than half of its natural sugar as fructose. Glucose is the other simple sugar found in these foods which helps absorb fructose in the small intestine. The more glucose than fructose in a food, the more “gut-friendly” it is. Avoid these high fructose foods: Fruit: apple, pear, Asian pear, watermelon, fig, mango, cherries Vegetables: artichokes, sugar snap peas, asparagus Sweeteners: honey, agave, high fructose corn syrup (HFCS), fruit juice concentrate of the above fruits, and large amounts of sucrose (white or brown sugar) UMHS Division of Gastroenterology - 1 - Beverages: juices of the fruit listed above, regular soda and drinks that are sweetened with fructose or HFCS, rum General guidelines: Read labels and avoid products that contain fructose, crystalline fructose, HFCS, honey, agave, and fruit juice concentrates (i.e. -
Appendix 3.1 Birth Defects Descriptions for NBDPN Core, Recommended, and Extended Conditions Updated March 2017
Appendix 3.1 Birth Defects Descriptions for NBDPN Core, Recommended, and Extended Conditions Updated March 2017 Participating members of the Birth Defects Definitions Group: Lorenzo Botto (UT) John Carey (UT) Cynthia Cassell (CDC) Tiffany Colarusso (CDC) Janet Cragan (CDC) Marcia Feldkamp (UT) Jamie Frias (CDC) Angela Lin (MA) Cara Mai (CDC) Richard Olney (CDC) Carol Stanton (CO) Csaba Siffel (GA) Table of Contents LIST OF BIRTH DEFECTS ................................................................................................................................................. I DETAILED DESCRIPTIONS OF BIRTH DEFECTS ...................................................................................................... 1 FORMAT FOR BIRTH DEFECT DESCRIPTIONS ................................................................................................................................. 1 CENTRAL NERVOUS SYSTEM ....................................................................................................................................... 2 ANENCEPHALY ........................................................................................................................................................................ 2 ENCEPHALOCELE ..................................................................................................................................................................... 3 HOLOPROSENCEPHALY............................................................................................................................................................. -
Biliary Atresia Splenic Malformation Syndrome: a Single Center Experience
Acta Medica 2019; 36 - 41 acta medica ORIGINAL ARTICLE Biliary Atresia Splenic Malformation Syndrome: A Single Center Experience 1 ABSTRACT Onder Ozden ,[MD] ORCID: 0000-0001-5683-204X Objective: Biliary atresia splenic malformation (BASM) syndrome which is a subgroup of BA is associated with situs inversus, intestinal malrotation, Seref Selcuk Kilic1 MD] ,[ polysplenia, preduodenal portal vein, interrupted vena cava, congenital por- ORCID: 0000-0002-1427-0285 tocaval shunts and cardiac anomalies. We aimed to report our experiences 1 in BASM management and association of CMV infection. Murat Alkan ,[MD] Materials and Methods: The data were collected retrospectively from med- ORCID: 0000-0001-5558-9404 ical records of patients treated in Cukurova University between 2005-2017. Sex, age, liver function tests, serological test results, BA types, surgical find- 2 Gokhan Tumgor ,[MD] ings, and mortality were noted. ORCID: 0000-0002-3919-002X Results: Fifty-nine BA patients were diagnosed in the study period. Seven of them were classified as BASM. The median age was 60 days (45-90 days) 1 Recep Tuncer ,[MD] with a female/male ratio of 3/4. The main complaint of all patients was jaun- ORCID: 0000-0003-4670-8461 dice. The jaundice of 6 patients began since birth and one began at 20 days- age. Median total/direct blood bilirubin levels were 9.6/5.4 mg/dL. Median 1,*Cukurova University Department of Pediatric values of liver function tests; ALT, AST, and GGT were 77 IU/L, 201 IU/L Surgery, Adana, Turkey and 607 IU/L respectively. Five of the patients showed positive results for anti-CMV Ig M. -
University of Cincinnati
UNIVERSITY OF CINCINNATI Date:___________________ I, _________________________________________________________, hereby submit this work as part of the requirements for the degree of: in: It is entitled: This work and its defense approved by: Chair: _______________________________ _______________________________ _______________________________ _______________________________ _______________________________ Prevalence of Subclinical Vitamin K Deficiency in Cholestatic Liver Disease A thesis submitted to the Division of Research and Advanced Studies of the University of Cincinnati In partial fulfillment of the Requirements for the degree of MASTER OF SCIENCE In the Division of Epidemiology and Biostatistics, Department of Environmental Health of the College of Medicine 2004 by Jennifer A. Strople, M.D. B.A., University of Virginia, 1994 M.D., University of Alabama at Birmingham School of Medicine, 1998 Thesis committee: James E. Heubi, M.D. (Chair and Advisor) Paul Succop, Ph.D. Abstract Current practice is to monitor prothrombin time as an indicator of vitamin K sufficiency in cholestatic liver disease. Since prothrombin time is a surrogate marker, it may underestimate the actual prevalence of vitamin K deficiency in this population. This study investigates the frequency of vitamin K deficiency among a convenience sample of children and adults with cholestatic liver disease by determining plasma levels of protein induced in vitamin K absence II (PIVKA-II), and assesses the relationship between PIVKA-II levels and markers of cholestasis, measured prothrombin time, and vitamin A, E and 25-hydroxyvitamin D levels. Methods: Subjects with cholestatic liver disease were recruited from the Cincinnati referral area. Subjects with decompensated cirrhosis, malignancy, concurrent disease that results in fat malabsorption and AIDS were excluded. All subjects had blood collected for liver function tests, prothrombin time (PT), INR, bile acids, 25-hydroxyvitamin D, vitamin A, vitamin E and PIVKA-II levels. -
Chronic Pancreatitis: Introduction
Chronic Pancreatitis: Introduction Authors: Anthony N. Kalloo, MD; Lynn Norwitz, BS; Charles J. Yeo, MD Chronic pancreatitis is a relatively rare disorder occurring in about 20 per 100,000 population. The disease is progressive with persistent inflammation leading to damage and/or destruction of the pancreas . Endocrine and exocrine functional impairment results from the irreversible pancreatic injury. The pancreas is located deep in the retroperitoneal space of the upper part of the abdomen (Figure 1). It is almost completely covered by the stomach and duodenum . This elongated gland (12–20 cm in the adult) has a lobe-like structure. Variation in shape and exact body location is common. In most people, the larger part of the gland's head is located to the right of the spine or directly over the spinal column and extends to the spleen . The pancreas has both exocrine and endocrine functions. In its exocrine capacity, the acinar cells produce digestive juices, which are secreted into the intestine and are essential in the breakdown and metabolism of proteins, fats and carbohydrates. In its endocrine function capacity, the pancreas also produces insulin and glucagon , which are secreted into the blood to regulate glucose levels. Figure 1. Location of the pancreas in the body. What is Chronic Pancreatitis? Chronic pancreatitis is characterized by inflammatory changes of the pancreas involving some or all of the following: fibrosis, calcification, pancreatic ductal inflammation, and pancreatic stone formation (Figure 2). Although autopsies indicate that there is a 0.5–5% incidence of pancreatitis, the true prevalence is unknown. In recent years, there have been several attempts to classify chronic pancreatitis, but these have met with difficulty for several reasons. -
Newborn Handbook
Pediatric Residency Newborn Handbook 2020-2021 1 Table of Contents Topic Page Contact Information 3 Routine Newborn Care 4 Discharge Talk Guidelines 5-7 AAP Recommendations for Healthy Term Newborn Discharge Criteria 8-9 Basic management of maternal labs/risk factors and Medication Refusal 10-11 Routine Vitamin K Prophylaxis 12 Hep B Vaccine Information and Management of Maternal Hepatitis B Status 13 Routine Erythromycin Prophylaxis for Ophthalmia Neonatorum 14 Hearing Screen 15 CCHD Screening 16 Michigan Newborn Screening 17 Breast Feeding 18-19 Infant feeding policy (donor breast milk) 20 Ankyloglossia and Frenotomy 21 Circumcision 22 Car Seat Safety 23-24 Nursery Protocols 25 Locating Policies, Procedures & Protocols 25 NRP (Neonatal Resuscitation Protocol), APGAR Scoring, MR. SOPA 26 Indirect Hyperbilirubinemia 27-30 Hypoglycemia Algorithm 31-32 Hypoglycemia Treatment, SGA & LGA cutoffs, and Pounds to Kilogram Conversion 33 Chorioamnionitis protocol and antibiotic duration, GBS Algorithm 34-35 Temperature Regulation 36-38 On-Call Problems & a note about SBARs 39 Respiratory/Cardiovascular Respiratory Distress 40-41 Cyanosis 42 Heart Murmurs, Cardiac Exam, and CHD 43 FEN/GI/Endo: Newborn Fluid Management and Weight Specific Guidelines for Feeding 44 Bilious Vomiting 45-46 When You’re Asked About the Appearance of Baby Poop 47 Bloody Stool 48 No stool in 48 hours of life and No void in 30 hours of life 49 Maternal Graves’ Disease 50 Renal Management of Antenatal Hydronephrosis 51 HEENT/Neuro Skull Sutures & Fontanels / Extracerebral Fluid Collections/Subgaleal Hemorrhage 52-53 Infant Fall 54 Oral-facial clefts 55-56 Neonatal Seizures 57 Neonatal Abstinence Syndrome 58-60 Infectious Disease Rubella, CMV, HIV 61 Syphillis 62-63 Toxoplasmosis, HSV 64 Recommended HSV management 64-67 Hepatitis C, Varicella 67 Assessing Gestational Age and the Ballard Score 68 Selected Lab Evaluation 69 Transferring to NICU 70 2 Contact Information Resident ASCOM: 76087.