DOCSLIB.ORG

Liver Disease

Total Page：16

File Type：pdf, Size：1020Kb

Download full-text PDF Read full-text
Liver Disease Practical Gastroenterology Liver Disease Wendy Blount, DVM Primary vs Secondary Liver Disease • One of the most difficult tasks of dealing with liver disease is to distinguish one from another • Primary Liver disease must be treated at it’s cause, if we are to do anything but palliate • Secondary Liver Disease just distracts us from the primary problem, whether liver disease, and/or something else • Secondary Liver Disease = Reactive Hepatopathy • However, as the liver acts as an innocent bystander, it can suffer collateral damage, which we should mediate Primary vs Secondary Liver Disease Signs of Primary Liver Disease – The Liver Is Sick! • Change in liver size or shape – Hepatomegaly – Microhepatia – Liver mass – Irregular liver margins – Radiographs the best test for liver size – Ultrasound the best test for liver shape and to find masses • Icterus without anemia • Hepatic encephalopathy Primary vs Secondary Liver Disease Signs of Primary Liver Disease – The Liver Is Sick! • Acholic feces • Superficial necrolytic hyperkeratosis – aka hepatocutaneous syndrome Primary vs Secondary Liver Disease Signs of Primary Liver Disease • Coagulopathy – The Liver Is Very Sick!! – GI hemorrhage death spiral 1. Increased stomach acidity – Decreased hepatic clearance of gastrin, decreased mucus, mucosal ischemia – Increased bile acids further stimulate HCl secretion 2. Clotting factor’s, AT3 not produced adequately by failing liver – Poor clotting and thromboembolic disease 3. DIC – more AT3 consumption – more thrombosis • Positive Feedback Loop #1 - GI Bleeding exacerbates hepatic encephalopathy • Positive Feedback Loop #2 - Large bleed can cause depletion coagulopathy Primary vs Secondary Liver Disease Signs of Primary Liver Disease • Coagulopathy – The Liver Is Very Sick!! – GI hemorrhage death spiral 4. Cholestasis Vit K absorption – Vit K needed to make factors 2,7,9,10 – Long term antimicrobial Tx inhibits Vit K production – Hemorrhage elsewhere only when near death • Petechiae, bruising, bleeding into cavities Normal Liver Size • Look at the gastric axis!! (fundus to pylorus) • Normal gastric axis range: • From: perpendicular to the spine; To: parallel to the ribs. Hepatomegaly • Look at the gastric axis!! (fundus to pylorus) • Gastric axis more acute angle with the spine Hepatomegaly • Focal hepatomegaly is often, but not always neoplasia • Gallbladder mass can be mistaken for liver mass or even normal liver if filled with amorphous sludge Hepatomegaly • Focal hepatomegaly is often, but not always neoplasia • Gallbladder mass can be mistaken for liver mass or even normal liver if filled with amorphous sludge Hepatomegaly • Focal hepatomegaly is often, but not always neoplasia • Gallbladder mass can be mistaken for liver mass or even normal liver if filled with amorphous sludge Hepatomegaly • Focal hepatomegaly is often, but not always neoplasia • Gallbladder mass can be mistaken for liver mass or even normal liver if filled with amorphous sludge • Glycogen vacuolar hepatopathy can be focal • Nodular hyperplasia is a benign process causing liver masses • Generalized hepatomegaly • Primary or secondary liver disease • Toxic exposure and swelling of the liver – hypoechoic large liver with smooth borders and rounded margins • Steroid hepatopathy – hyperechoic large liver with smooth borders and normal margins • Any generally large liver with rounded margins can be neoplastic, regardless of echotexture and relative echogenicity – LSA, MCT, hepatocellular carcinoma Microhepatia - Microhepatica • Look at the gastric axis!! (fundus to pylorus) • Gastric axis more obtuse angle with the spine Microhepatia - Microhepatica Differential Diagnosis: 1. Atrophy • Portasystemic shunt – any age, chronic disease • AV fistula – usually less than 2 yrs old (rare) • Often normal panel, but elevated bile acids + ammonias Microhepatiacirrhosis - Microhepatica scites Differential Diagnosis: iver 1. Atrophy – Small, maybe hyperechoic liver • Portasystemic shunt – any age, chronic disease • AV fistula – usually less than 2 yrs old (rare) • Often normal panel, but elevated bile acids + ammonias 2. Cirrhosis • history of chronic inflammatory disease • History of massive hepatic necrosis • History of untreated PSS for years • Usually middle aged to older patients • Liver enzymes usually but not always elevated, bile acids elevated, albumin may be low • Ascites is common Microhepatia - Microhepatica Differential Diagnosis: 3. Congenital small size – diagnosis of exclusion • Imaging – no lumps, normal appearing margins, normal echotexture (just small) • All liver blood tests normal, except changes due to secondary liver disease • Liver biopsy normal • Splenic portagram normal Problems Causing Reactive Hepatopathy • Any systemic infection or inflammation – Occult - urinary tract, metritis, prostatitis, dental disease, etc. • Disease of organ drained by portal vein – Gut, spleen, pancreas (ultrasound the best test) • Hyperthyroidism in cats • Severe muscle disease • Untreated Cushing’s Disease or diabetes mellitus • Hypoxia, passive congestion – heart failure, respiratory disease, severe anemia – hypotension • Drugs causing liver enzyme induction • VARYING DEGREES OF COLLATERAL DAMAGE Diagnostics for Liver Disease First Tier Tests: • CBC, panel, urinalysis • Fecal flotation, direct smear, cytology • HWAg in the dog, FeLV/FIV in the cat Second Tier Tests: • Bile Acids • Complete Abdominal Ultrasound (not focused liver/GB and not GlobalFAST®) • Liver sampling & spleen FNA – after BMBT • FNA – 25-50% diagnostic • Ultrasound guided biopsy – 50% diagnostic • Wedge biopsy – more likely to be diagnostic Diagnostics for Liver Disease Third Tier Tests: • if Bile Acids are equivocal and PSS is suspected – Ammonia tolerance test – Splenic portagram • Coagulation tests if significantly systemically ill, severe liver disease is suspected, or liver sampling will be done • Hunt for causes of secondary liver disease • Imaging – thoracic rads and ultrasound • Testing for infectious disease and Cushing’s Disease • Diagnostic surgery, MRI/CT Pattern Recognition - Liver Disease • High liver enzymes – ALT – hepatocellular disease (hepatocellular membrane) – ALKP, GGT – more sensitive indicator of cholestasis than bilirubin (from biliary epithelium) • Low albumin – low alb indicates severe disease (90% loss of hepatic function) – Beware human labs (falsely low values) - Abaxxis • Low fasting glucose (70% loss of hepatic function) • High prost-prandial glucose • Low BUN • Abnormal cholesterol, triglycerides ALT Gems The enzyme formerly known as SGPT • RBC & skeletal muscle contain small amts of ALT – Hemolysis and muscle damage cause moderate elevations in ALT (2-3x) • ALT - t1/2 – 1-2 days – the first to improve – Starts improving within 1-2 weeks of liver disease resolution – remains elevated during hepatic regeneration • DDx hepatocellular turnover – Hepatocellular disease – Poor perfusion – hypotension, passive congestion, anemia • Marker for anaphylaxis, along with increased PTT and AFAST sonogram – Trauma – surgery, trauma, peritonitis – Toxicity ALKP Gems • Bone disease can also cause ALKP elevation – Neoplasia, osteomyelitis, hyperPTH, growth & healing • DDx cholestasis: – Gallbladder disease • Intramural - Gallbladder sludge, mucocoele, stones, infection • Mural - Gallbladder wall polyps, masses, infection • Extramural – Pancreatitis, abdominal mass, duodenal foreign body, diaphragmatic hernia • Gallbladder trauma or rupture – Liver Disease • microscopic cholestatic disease (infectious, inflammatory, toxic), liver trauma, neoplasia, lipidosis ALKP Gems • DDx cholestasis: – Systemic cholestatic disease • Endocrine – diabetes mellitus, hyperadrenocorticism, chronic stress, hyperthyroidism • Passive congestion – RHF, pericardial disease • Systemic infection/inflammation – especially sepsis, rickettsial disease, protozoal disease, fungal disease GGT Gems • less influenced by secondary liver disease or acute hepatic necrosis than ALKP • Not confounded by bone disease as ALKP • more useful in cats to identify fatty liver • More useful in dogs to identify biliary disease • Otherwise, follows ALKP Hepatotoxic Drugs • Drug induced ALKP does not necessarily indicate hepatic pathology – Assess with bile acids if concerned – Phenobarbital can also cause hepatotoxicity, but glucocorticoids almost never do • GGT increases markedly with corticosteroid enzyme induction in dogs • Drug induced ALKP takes weeks to months to resolve after stopping the drug • Glucocorticoids almost always elevate ALKP (and ALT) in dogs, but almost never in cats Hepatotoxic Drugs • Drugs that increase ALT are more likely to cause hepatic damage if continued • At a marked increase in ALT, the offending drug should ideally be stopped, and ALT rechecked in 30 days • Lomustine - missing from the chart, but can cause potentially life threatening hepatotoxicity indicated first by increased ALT and then decreased albumin • Use of any drug on either chart indicates monitoring of liver nz + bile acids, albumin Elevated Liver Enzymes in the Cat Cats are not little dogs – Thumb Rules • Cats with persistently elevated enzymes should be worked up – T1/2 of liver enzymes is hours in the cat, not days as in the dog – cats have 1/3 the liver ALKP compared to dogs • Cats with significant cholangiohepatitis can have normal liver enzymes, though that problem often causes very high ALT • GGT in cats has higher sensitivity but lower specificity for biliary disease • If a cat has biliary disease, GGT is likely high • If cat does
Load more

Recommended publications
  • Management of Liver Complications in Sickle Cell Disease
    | MANAGEMENT OF SICKLE CELL DISEASE COMPLICATIONS BEYOND ACUTE CHEST | Management of liver complications in sickle cell disease Abid R. Suddle Institute of Liver Studies, King’s College Hospital, London, United Kingdom Downloaded from https://ashpublications.org/hematology/article-pdf/2019/1/345/1546038/hem2019000037c.pdf by DEUSCHE ZENTRALBIBLIOTHEK FUER MEDIZIN user on 24 December 2019 Liver disease is an important cause of morbidity and mortality in patients with sickle cell disease (SCD). Despite this, the natural history of liver disease is not well characterized and the evidence basis for specific therapeutic intervention is not robust. The spectrum of clinical liver disease encountered includes asymptomatic abnormalities of liver function; acute deteriorations in liver function, sometimes with a dramatic clinical phenotype; and decompensated chronic liver disease. In this paper, the pathophysiology and clinical presentation of patients with acute and chronic liver disease will be outlined. Advice will be given regarding initial assessment and investigation. The evidence for specific medical and surgical interventions will be reviewed, and management recommendations made for each specific clinical presen- tation. The potential role for liver transplantation will be considered in detail. S (HbS) fraction was 80%. The patient was managed as having an Learning Objectives acute sickle liver in the context of an acute vaso-occlusive crisis. • Gain an understanding of the wide variety of liver pathology Treatment included IV fluids, antibiotics, analgesia, and exchange and disease encountered in patients with SCD blood transfusion (EBT) with the aim of reducing the HbS fraction • Develop a logical approach to evaluate liver dysfunction and to ,30% to 40%. With this regimen, symptoms and acute liver dys- disease in patients with SCD function resolved, but bilirubin did not return to the preepisode baseline.
    [Show full text]
  • Accuracy and Reliability of Palpation and Percussion for Detecting Hepatomegaly: a Rural Hospital-Based Study
    Accuracy and reliability of palpation and percussion for detecting hepatomegaly: a rural hospital-based study Rajnish Joshi, Amandeep Singh, Namita Jajoo, Madhukar Pai,* S P Kalantri Department of Medicine, Mahatma Gandhi Institute of Medical Sciences, Sevagram 442 102, Maharashtra; and *Division of Epidemiology, University of California at Berkeley, Berkeley, CA 94720, USA Background: Palpation and percussion are standard Although many physicians believe that physical bedside techniques used to diagnose hepatomegaly. examination can accurately identify hepatomegaly, some Ultrasonography is a noninvasive and accurate method published reports suggest that physical signs lack accu- for measurement of liver size, but many patients in racy and reliability.1,2,3 To our knowledge, no study from developing countries have limited access to it. We India has evaluated the accuracy of physical examina- compared the accuracy of palpation and percussion tion in the assessment of enlarged liver. We conducted in a rural population in central India, using this study to determine how accurately doctors can ultrasonography as a reference standard. Methods: distinguish an enlarged liver from a normal sized one, The study design was a blinded, cross-sectional analysis and how often they agree with one another while as- of a hospital-based case series. Three physicians, sessing liver size. blind to clinical data and to each others results, independently used palpation and percussion to detect Methods hepatomegaly. Diagnostic accuracy was measured by We enrolled consecutive patients admitted to the Medi- computing sensitivity, specificity, and likelihood ratio cine wards between February 1 and 15, 2003. Patients values. Inter-physician agreement was assessed using with pleural diseases (effusion or pneumothorax) or the kappa statistic.
    [Show full text]
  • Stauffer's Syndrome
    Published online: 2021-06-17 Case Report Stauffer’s Syndrome: A Rare Paraneoplastic Syndrome with Renal Cell Carcinoma Abstract Mayank Jain, An elderly male patient presented with cholestatic jaundice and weight loss. On evaluation, he was Joy Varghese1, found to have left renal mass and hepatomegaly. Diagnosis of Stauffer’s syndrome was confirmed K M based on his clinical history, biochemical evaluation, and liver biopsy. Resolution of jaundice was 2 noted after removal of the renal mass. Muruganandham , Jayanthi Keywords: Cholestasis, jaundice, paraneoplastic, renal Venkataraman Departments of Gastroenterology, 1Hepatology Introduction mild portal fibrosis with hepato canalicular and 2Urology, Gleneagles bilirubinomatosis and lobular inflammation. Nonmetastatic nephrogenic hepatic Global Health City, Chennai, The possibility of cholestatic jaundice due Tamil Nadu, India dysfunction syndrome (Stauffer’s syndrome) to paraneoplastic manifestation of renal cell is a paraneoplastic manifestation that often carcinoma was considered. The patient was appears as the initial clinical presentation managed by therapeutic plasma exchange of renal cell carcinoma, bronchogenic for severe pruritus in the preoperative carcinoma, leiomyosarcoma, and prostate period, and he underwent laparoscopic adenocarcinoma.[1-3] Although jaundice has left radical nephrectomy. The surgical rarely been described with this syndrome, a specimen showed clear cell renal cell few case reports have highlighted a variant carcinoma with perinephric fat, hilar sinus of the syndrome with deep icterus.[4,5] fat, hilar vessels, and ureter free of tumor Case Report invasion (Fuhrman Nuclear Grade II). Postoperatively, he had gradual fall in the A 63-year-old male presented with a history bilirubin values over a period of 4 weeks. of yellowish discoloration of eyes and urine, generalized itching, and weight loss Discussion of 10 kg over last 1 month.
    [Show full text]
  • Stem Cell Transplantation for Congenital Dyserythropoietic Anemia
    LETTERS TO THE EDITOR ment has also been successfully used in patients with Stem cell transplantation for congenital CDA type I,6,7 whereas splenectomy has been proved to dyserythropoietic anemia: an analysis from the reduce the number of transfusions in CDA II.8 European Society for Blood and Marrow Overall, the prognosis of CDA patients is good;2 how- Transplantation ever, stem cell transplantation (SCT) represents the only curative option for this disease. Some reports have Congenital dyserythropoietic anemias (CDA) are a shown its efficacy, but data from the literature are scarce group of heterogeneous disorders characterized by and limited to a very small number of patients, mostly hyporegenerative anemia and ineffective erythropoiesis, transplanted from sibling donors.9-16 with related reticulocytopenia, and iron overload.1 In this retrospective study, we describe the outcome of Specific morphological aspects of late erythroblasts in the SCT in a large cohort of patients with CDA. The study bone marrow form the most important, albeit non-specif- was conducted on behalf of the Severe Aplastic Anemia ic, feature of the disease. Morphology has always been Working Party (SAAWP) of the European Society for the most important tool for diagnosis and is still used to Bone and Marrow Transplantation (EBMT) and relied on classify the disease into three classical forms and data from patients affected with CDA who underwent variants.2 Nonetheless, in the last few years, mutations of SCT and were registered in the EBMT Database. Clinical six specific genes related to the regulation of DNA and information on the disease was collected by a question- cell division have been identified as being causative.3-5 naire distributed to participating centers and the details Patients with CDA usually show anemia, jaundice, on transplant procedures were obtained by analyzing the splenomegaly, ineffective erythropoiesis and the typical database.
    [Show full text]
  • Pulmonary Vascular Complications of Liver Disease
    American Thoracic Society PATIENT EDUCATION | INFORMATION SERIES Pulmonary Vascular Complications of Liver Disease People who have advanced liver disease can have complications Jaundice that affect the heart and lungs. It is not unusual for a person (yellow tint to skin with severe liver disease to have shortness of breath. Breathing and eyes) problems can occur because the person can’t take as big a breath due to large amounts of ascites (fluid in the abdomen) or pleural effusions (fluid build-up between the tissues that line the lung and chest) or a very large spleen and liver that pushes the diaphragm up. Breathing problems can also occur with Hepatomegaly liver disease from changes in the blood vessels and blood flow in the lungs. There are two well-recognized conditions that can result from liver disease: hepatopulmonary syndrome and portopulmonary hypertension. This fact sheet will review these Breathing two conditions and how they relate to liver disease. problems What is liver disease? the rest of your body. These toxins can damage blood vessels The liver is the second largest organ in the body and has many in your lungs leading to dilated (enlarged) or constricted important roles within the body including helping with digestion, (narrowed) vessels. Two different conditions can be seen in the metabolizing drugs, and storing nutrients. Its main job is to lungs that arise from liver disease: hepatopulmonary syndrome filter blood coming from the digestive tract and remove harmful and portopulmonary hypertension: CLIP AND COPY AND CLIP substances from it before passing it to the rest of the body.
    [Show full text]
  • Parasites in Liver & Biliary Tree
    Parasites in Liver & Biliary tree Luis S. Marsano, MD Professor of Medicine Division of Gastroenterology, Hepatology and Nutrition University of Louisville & Louisville VAMC 2011 Parasites in Liver & Biliary Tree Hepatic Biliary Tree • Protozoa • Protozoa – E. histolytica – Cryptosporidiasis – Malaria – Microsporidiasis – Babesiosis – Isosporidiasis – African Trypanosomiasis – Protothecosis – S. American Trypanosomiasis • Trematodes – Visceral Leishmaniasis – Fascioliasis – Toxoplasmosis – Clonorchiasis • Cestodes – Opistorchiasis – Echynococcosis • Nematodes • Trematodes – Ascariasis – Schistosomiasis • Nematodes – Toxocariasis – Hepatic Capillariasis – Strongyloidiasis – Filariasis Parasites in the Liver Entamoeba histolytica • Organism: E. histolytica is a Protozoa Sarcodina that infects 1‐ 5% of world population and causes 100000 deaths/y. – (E. dispar & E. moshkovskii are morphologically identical but only commensal; PCR or ELISA in stool needed to differentiate). • Distribution: worldwide; more in tropics and areas with poor sanitation. • Location: colonic lumen; may invade crypts and capillaries. More in cecum, ascending, and sigmoid. • Forms: trophozoites (20 mcm) or cysts (10‐20 mcm). Erytrophagocytosis is diagnostic for E. histolytica trophozoite. • Virulence: may increase with immunosuppressant drugs, malnutrition, burns, pregnancy and puerperium. Entamoeba histolytica • Clinical forms: – I) asymptomatic; – II) symptomatic: • A. Intestinal: – a) Dysenteric, – b) Nondysenteric colitis. • B. Extraintestinal: – a) Hepatic: i) acute
    [Show full text]
  • Hypereosinophilic Syndrome: Clinical, Laboratory, and Imaging Manifestations in Patients with Hepatic Involvement
    대 한 방 사 선 의 학 회 지 1993 ; 29 (4) : 757~ 764 Journal of Korean Radiological Society, July, 1993 Hypereosinophilic Syndrome: Clinical, Laboratory, and Imaging Manifestations in Patients with Hepatic Involvement Gi Beom Kim, M.D., Ok Hwoa Kim, M.D.*, Jong Min Lee, M.D., Yeong Soon Sung, M.D., Duk Sik Kang, M.D. Department 01 Radiology, Kyungpook National Universi낀I College 01 Medicine - Abstract- The hypereosinophilic syndrome (HES) commonly involves liver and spleen but only a few literature has reported the imaging features. In this article, we present the imaging features of the liver and spleen in HES patients together with clinical and laboratoη features. This study included 5 HES patients with hepatic involvement. Extensive laboratory tests including multiple hematologic, serolo밍 c ， parasitologic, and immunologic examinations were performed. Imaging studies includ­ ed CT, ultrasound (US) of upper abdomen and hepatosplenic scintigraphy. All patients were perio벼C 떠 lyexam­ ined by laboratory and imaging studies for 4 to 24 months. The common clinical presentations were weakness, rnild fever, and dry cough. All patients revealed leukocy­ tosis with eosinophilia of 40 to 80% and benign eosnophilic hyperplasia of the bone marrow. The percutane­ ous biopsy of the hepatic focal lesions performed in 2 patients showed numerous benign eosinophilic infIltrates and one of them revealed combined centrilobular necrosis of hepatocytes. All cases revealed hepatomegaly with m띠 tiple focallesions on at least one of CT, US, or scintigraphy. These findings completely disappeared in 2 to 6 months following medication of corticosteroid or antihistamines. The HES involved the liver and CT, US , or scintigraphic studies showed hepatic multifocal lesions with hepatomegaly.
    [Show full text]
  • A 7 Year Old Girl with Anemia and Massive Hepatosplenomegaly Mohammad Mizanur Rahman, Md
    | Case Presentation | No. 01-2018 | A 7 year old girl with anemia and massive hepatosplenomegaly Mohammad Mizanur Rahman, Md. Mehedhi Hasan Shourov, Debashish Saha, Md. Abdul Ali Miah and S. M. Motahar Hossain Article Info Presentation of Case advised to get her admitted into the children ward for further management. In the child Departet of Heatology, Ared Dr. Md. Mehedhi Hasan Shourov: A 7 year old ward, patient and her parents were thoroughly Fores Istitute of Pathology, Dhaka Catoet, Dhaka, Bagladesh MMR, girl reported to the child outpatient department interviewed and the child was re-examined. MMHS; Departet of Bioheistry, of a military hospital in Chittagong (South-East The indoor physician ordered initial routine Dhaka Catoet, Dhaka, Bagladesh part of Bangladesh) Cantonment with the com- investigations such as complete blood count, DS; Departet of Mediie, Co- plaints of generalized weakness, loss of appe- peripheral blood film examination, malaria ied Military Hospital, Dhaka, Bagla- desh MAAM, SMMH tite, gradual distention of the abdomen and parasite, immuno-chromatographic test for weight loss. malaria, random blood sugar, liver function tests, urine routine and microscopic examina- The child was reasonably well and performing tion. After getting the results of all investi- For Correspondene: all her daily activities at her own 1 year before. Mohaad Mizaur Raha gations, child specialists sat together, reviewed She was also going to the school regularly and [email protected] her history, physical findings and the results of was worried when her parents noticed the all investigations so far received, discussed the Reeied: Otoer distension of her abdomen and reluctant to take case in details and decided to refer the patient Aepted: Jauary food adequately.
    [Show full text]
  • 1 a Clinical Approach to Inherited Metabolic Diseases
    1 A Clinical Approach to Inherited Metabolic Diseases Jean-Marie Saudubray, Isabelle Desguerre, Frédéric Sedel, Christiane Charpentier Introduction – 5 1.1 Classification of Inborn Errors of Metabolism – 5 1.1.1 Pathophysiology – 5 1.1.2 Clinical Presentation – 6 1.2 Acute Symptoms in the Neonatal Period and Early Infancy (<1 Year) – 6 1.2.1 Clinical Presentation – 6 1.2.2 Metabolic Derangements and Diagnostic Tests – 10 1.3 Later Onset Acute and Recurrent Attacks (Late Infancy and Beyond) – 11 1.3.1 Clinical Presentation – 11 1.3.2 Metabolic Derangements and Diagnostic Tests – 19 1.4 Chronic and Progressive General Symptoms/Signs – 24 1.4.1 Gastrointestinal Symptoms – 24 1.4.2 Muscle Symptoms – 26 1.4.3 Neurological Symptoms – 26 1.4.4 Specific Associated Neurological Abnormalities – 33 1.5 Specific Organ Symptoms – 39 1.5.1 Cardiology – 39 1.5.2 Dermatology – 39 1.5.3 Dysmorphism – 41 1.5.4 Endocrinology – 41 1.5.5 Gastroenterology – 42 1.5.6 Hematology – 42 1.5.7 Hepatology – 43 1.5.8 Immune System – 44 1.5.9 Myology – 44 1.5.10 Nephrology – 45 1.5.11 Neurology – 45 1.5.12 Ophthalmology – 45 1.5.13 Osteology – 46 1.5.14 Pneumology – 46 1.5.15 Psychiatry – 47 1.5.16 Rheumatology – 47 1.5.17 Stomatology – 47 1.5.18 Vascular Symptoms – 47 References – 47 5 1 1.1 · Classification of Inborn Errors of Metabolism 1.1 Classification of Inborn Errors Introduction of Metabolism Inborn errors of metabolism (IEM) are individually rare, but collectively numerous.
    [Show full text]
  • A Study of Hepatobiliary Involvement in Adult Patients with Sickle Cell Disease
    International Journal of Advances in Medicine Mohanty AP et al. Int J Adv Med. 2020 Sep;7(9):1361-1366 http://www.ijmedicine.com pISSN 2349-3925 | eISSN 2349-3933 DOI: http://dx.doi.org/10.18203/2349-3933.ijam20203599 Original Research Article A study of hepatobiliary involvement in adult patients with sickle cell disease Ambika Prasad Mohanty, Venkatesh Yellapu, Kanduri Manoj Kumar, Akshay Saxena, Dandi Suryanarayana Deo* Department of General Medicine, Kalinga Institute of Medical Sciences, KIIT Deemed to be University, Bhubaneswar, Odisha, India Received: 23 June 2020 Accepted: 30 July 2020 *Correspondence: Dr. Dandi Suryanarayana Deo, E-mail: [email protected] Copyright: © the author(s), publisher and licensee Medip Academy. This is an open-access article distributed under the terms of the Creative Commons Attribution Non-Commercial License, which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited. ABSTRACT Background: Sickle cell disease (SCD) encompasses a group of hemoglobinopathies characterized by a single amino acid substitution in the ß-globin chain. Hepatobiliary complications are frequent among sickle cell disease patients. Sickle cell disease has been extensively studied. However, data about hepatobiliary abnormalities among the adult age group are limited. Aim of our study aims to find the prevalence of hepatic-biliary involvement in Sickle cell disease in adult patients admitted to our hospital. Methods: A prospective study was done for a period of two years from October 2017 to October 2019. Subjects of both sexes above the age of 18 years with SCD admitted to our hospital were enrolled.
    [Show full text]
  • Hepatomegaly and Multiple Liver Lesions
    Self-assessment corner 439 1 Litovitz TL, Schmitz BF, Bailey KM. 1989 Annual report of 2 Krause DS, Wolf BA, Shaw LM. Acute aspirin overdose: the American Association of Poison Control Centres mechanisms oftoxicity. Ther Drug Monitor 1992;14:441-51. National Data Collection System. Am J Emerg Med 3 Temple AR. Acute and chronic effects ofaspirin toxicity and 1990;8:394-442. their treatment. Arch Intern Med 1981;141:364-9. Postgrad Med J: first published as 10.1136/pgmj.74.873.439 on 1 July 1998. Downloaded from Hepatomegaly and multiple liver lesions Ho-Choong Chang, Ba Nguyen, Fintan Regan A 33-year-old man was referred for possible liver transplantation. The patient was initially diag- nosed at birth when he presented with an enlarged liver and episodes of hypoglycaemia. A liver biopsy at the time showed pale hepatic cells by virtue of cytoplasmic granularity and periportal nuclear ballooning (figure 1). He was treated initially with dietary modifications but subsequently required night time dextrose and corn starch. Failed medical therapy prompted referral for liver transplant evaluation. Physical examination showed massive hepatomegaly. Liver function tests were abnormal with a significantly raised alkaline phosphatase and transaminase. Sonography showed hepatomegaly with multiple focal lesions unchanged in size since ultrasound 3 years ear- lier. Computed tomography (CT) showed multiple well-defined low-attenuation lesions throughout the liver. The largest of these measured 8 x 8 x 4 cm and contained foci of coarse cal- cification (figure 2). ~~~'-^ ~.:. I @=,S v.. ip http://pmj.bmj.com/ Imaging Department, Johns Hopkins Bayview Medical Centre, Johns Hopkins Figure 1 Liver biopsy showing pale hepatic cells Figure 2 Axial CT scan showed multiple University Medical well-defined low-attenuation lesions throughout the School, Baltimore, MD liver 21224-2780, USA on October 1, 2021 by guest.
    [Show full text]
  • Intractable Mechanical Hemolytic Anemia Complicating Mitral Valve
    Wang et al. BMC Cardiovascular Disorders (2020) 20:104 https://doi.org/10.1186/s12872-020-01382-8 RESEARCH ARTICLE Open Access Intractable mechanical hemolytic anemia complicating mitral valve surgery: a case series study Jin Wang1, Hanlin Zhang2, Hongyang Fan3, Kang Chen2, Yuelun Zhang1, Kaicheng Song1, Hushan Ao4* and Chunhua Yu1* Abstract Background: Intractable, mechanical hemolytic anemia (IMHA) is a rare catastrophic complication following mitral valve surgery. We analyzed patient characteristics and IMHA management by reoperations after mitral valve surgery. Methods: We collected medical records from mitral valve patients requiring reoperation due to IMHA. Inclusion criteria: hemoglobin < 100 g/L; positive hemolysis tests and echocardiography results; and exclusion of other hemolysis causes. Results: Data from 25 IMHA cases included 10 (40%) early onset (1.3 (0.3,3.0) months) and 15 (60%) late onset (120 (24,204) months) cases. Early IMHA etiologies included surgical defects (6, 60%), uncontrolled infection (3, 30%) and Bechet’s disease (1, 10%). Late IMHA etiologies included degeneration (13, 87%), new infection (1, 7%) and trauma (1, 7%). There were more mechanical valves (15, 88%) than bio-valves (2, 12%); the main valvular dysfunction was paravalvular leak (16, 64%). IMHA manifestations included jaundice (18, 72%), dark urine (21, 84%), heart failure (16, 64%), acute kidney injury (11, 44%), hepatomegaly (15, 60%), splenomegaly (15, 60%) and pancreatitis (1, 4%). Laboratory results showed decreased hemoglobin (70 ± 14 g/L) and increased bilirubin (72 ± 57 μmol/L), lactate dehydrogenase (2607 ± 2142 IU/L) and creatinine (136 ± 101 μmol/L) levels. Creatinine level negatively correlated with hemoglobin level (B = -3.33, S.E.
    [Show full text]