Progress Report Cholestasis and Lesions of the Biliary Tract in Chronic Pancreatitis
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A Drug-Induced Cholestatic Pattern
Review articles Hepatotoxicity: A Drug-Induced Cholestatic Pattern Laura Morales M.,1 Natalia Vélez L.,1 Octavio Germán Muñoz M., MD.2 1 Medical Student in the Faculty of Medicine and Abstract the Gastrohepatology Group at the Universidad de Antioquia in Medellín, Colombia Although drug induced liver disease is a rare condition, it explains 40% to 50% of all cases of acute liver 2 Internist and Hepatologist at the Hospital Pablo failure. In 20% to 40% of the cases, the pattern is cholestatic and is caused by inhibition of the transporters Tobon Uribe and in the Gastrohepatology Group at that regulate bile synthesis. This reduction in activity is directly or indirectly mediated by drugs and their me- the Universidad de Antioquia in Medellín, Colombia tabolites and/or by genetic polymorphisms and other risk factors of the patient. Its manifestations range from ......................................... biochemical alterations in the absence of symptoms to acute liver failure and chronic liver damage. Received: 30-01-15 Although there is no absolute test or marker for diagnosis of this disease, scales and algorithms have Accepted: 26-01-16 been developed to assess the likelihood of cholestatic drug induced liver disease. Other types of evidence are not routinely used because of their complexity and cost. Diagnosis is primarily based on exclusion using circumstantial evidence. Cholestatic drug induced liver disease has better overall survival rates than other patters, but there are higher risks of developing chronic liver disease. In most cases, the patient’s condition improves when the drug responsible for the damage is removed. Hemodialysis and transplantation should be considered only for selected cases. -
A Rare Hepatic Manifestation of Systemic Lupus Erythematosus
Cholestatic hepatitis in SLE Cholestatichepatitis:ararehepaticmanifestationof systemiclupuserythematosus WHChow,MSLam,WKKwan,WFNg Systemic lupus erythematosus is a multi-system inflammatory disease. The clinical manifestations are diverse. Hepatic manifestation is a rarely seen complication of systemic lupus erythematosus. We report a case of complication of systemic lupus erythematosus presenting as cholestatic hepatitis in a 56-year- old Chinese woman. The cholestatic hepatitis progressed as part of the lupus activity and responded to steroid therapy. HKMJ 1997;3:331-4 Key words: Hepatitis; Cholestasis; Lupus erythematosus, systemic; Liver Introduction of body weight and had had a poor appetite. She was a non-drinker and had no long term drug history. Systemic lupus erythematosus (SLE) is a multi- system inflammatory disease associated with the A general examination showed her to be jaundiced, development of auto-antibodies to a variety of self- pale, and dyspnoeic with an elevated body tempera- antigens. The clinical manifestations of SLE are ture of 38.2°C. Chest examination demonstrated coarse diverse. In 1982, the American Rheumatism Associa- crackles heard over both lung fields. Other parts of tion (ARA) published revised criteria for the classifi- the examination were unremarkable. There was 2+ cation of SLE.1 For a diagnosis of SLE, individuals proteinuria in the mid-stream urine but the culture should have four or more of the following features: for organisms was negative. Investigations revealed a malar rash, discoid rash, photosensitivity, oral ulcers, normochromic, normocytic anaemia (haemoglobin 9.4 non-erosive arthritis, pleuritis or pericarditis, renal g/dL [normal range, 11.5-15.5 g/dL]) with normal disorder, seizures or psychosis, haematological white cell and differential counts. -
Primary Biliary Cholangitis: a Brief Overview Justin S
REVIEW Primary Biliary Cholangitis: A Brief Overview Justin S. Louie,* Sirisha Grandhe,* Karen Matsukuma,† and Christopher L. Bowlus* Primary biliary cholangitis (PBC), previously referred to supported by the higher concordance of PBC in monozy- as primary biliary cirrhosis, is the most common chronic gotic compared with dizygotic twins.4 In addition, certain cholestatic autoimmune disease affecting adults in the human leukocyte antigen haplotypes have been associ- United States.1 It is characterized by a hallmark serologic ated with PBC, as well as variants at loci along the inter- signature, antimitochondrial antibody (AMA), and specific leukin-12 (IL-12) immunoregulatory pathway (IL-12A and bile duct pathology with progressive intrahepatic duct de- IL-12RB2 loci).5 struction leading to cholestasis. PBC is potentially fatal and can have both intrahepatic and extrahepatic complications. PATHOGENESIS EPIDEMIOLOGY The primary disease mechanism in PBC is thought to be T cell lymphocyte–mediated injury against intralobu- PBC affects all races and ethnicities; however, it is best lar biliary epithelial cells. This causes progressive destruc- studied in the Caucasian population. The condition pre- tion and eventual disappearance of the intralobular bile dominantly affects women older than 40 years, with a ducts. Molecular mimicry has been proposed as the ini- female/male ratio of 9:1.2 Although the incidence of PBC tiating event in the loss of tolerance primarily to mito- appears to be stable, the overall prevalence of the disease chondrial pyruvate dehydrogenase complex, E2, during is increasing.3 An individual’s genetic susceptibility, epige- which exogenous antigens evoke an immune response netic factors, and certain environmental triggers seem to that recognizes an endogenous (self) antigen inciting an play important roles. -
An Update on the Management of Cholestatic Liver Diseases
Clinical Medicine 2020 Vol 20, No 5: 513–6 CME: HEPATOLOGY An update on the management of cholestatic liver diseases Authors: Gautham AppannaA and Yiannis KallisB Cholestatic liver diseases are a challenging spectrum of autoantibodies though may be performed in cases of diagnostic conditions arising from damage to bile ducts, leading to doubt, suspected overlap syndrome or co-existent liver pathology build-up of bile acids and inflammatory processes that cause such as fatty liver. injury to cholangiocytes and hepatocytes. Primary biliary cholangitis (PBC) and primary sclerosing cholangitis (PSC) are ABSTRACT the two most common cholestatic disorders. In this review we Key points detail the latest guidelines for the diagnosis and management of patients with these two conditions. Ursodeoxycholic acid can favourably alter the natural history of primary biliary cholangitis in a majority of patients, given at the appropriate dose of 13–15 mg/kg/day. Primary biliary cholangitis Primary biliary cholangitis (PBC) is a chronic autoimmune liver Risk stratification is of paramount importance in the disorder characterised by immune-mediated destruction of management of primary biliary cholangitis to identify epithelial cells lining the intrahepatic bile ducts, resulting in patients with suboptimal response to ursodeoxycholic acid persistent cholestasis and, in some patients, a progression to and poorer long-term prognosis. Alkaline phosphatase cirrhosis if left untreated. The exact mechanisms remain unclear >1.67 times the upper limit of normal and a bilirubin above but are most likely a result of exposure to environmental factors in the normal range indicate high-risk disease and suboptimal a genetically susceptible individual.1 The majority of patients are treatment response. -
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. -
Diagnosis and Management of Primary Sclerosing Cholangitis
AASLD PRACTICE GUIDELINES Diagnosis and Management of Primary Sclerosing Cholangitis Roger Chapman,1 Johan Fevery,2 Anthony Kalloo,3 David M. Nagorney,4 Kirsten Muri Boberg,5 Benjamin Shneider,6 and Gregory J. Gores7 Preamble classification used by the Grading of Recommendation This guideline has been approved by the American Asso- Assessment, Development, and Evaluation (GRADE) ciation for the Study of Liver Diseases and represents the workgroup with minor modifications (Table 1).3 The position of the Association. These recommendations pro- strength of recommendations in the GRADE system are vide a data-supported approach. They are based on the classified as strong (class 1) or weak (class 2). The quality following: (1) formal review and analysis of the recently- of evidence supporting strong or weak recommendations published world literature on the topic (Medline search); is designated by one of three levels: high (level A), mod- (2) American College of Physicians Manual for Assessing erate (level B), or low-quality (level C). Health Practices and Designing Practice Guidelines1; (3) guideline policies, including the AASLD Policy on the Definition and Diagnosis Development and Use of Practice Guidelines and the Definitions. Primary sclerosing cholangitis (PSC) is a American Gastroenterological Association Policy State- chronic, cholestatic liver disease characterized by inflam- ment on Guidelines2; and (4) the experience of the au- mation and fibrosis of both intrahepatic and extrahepatic thors in the specified topic. bile ducts,4 leading to the formation of multifocal bile Intended for use by physicians, these recommenda- duct strictures. PSC is likely an immune mediated, pro- tions suggest preferred approaches to the diagnostic, ther- gressive disorder that eventually develops into cirrhosis, apeutic and preventative aspects of care. -
Cholestasis in Pediatrics
Review articles Cholestasis in Pediatrics Mónica D’Amato G., MD,1 Patricia Ruiz N., MD,2 Karen Aguirre R., MD.,2 Susana Gómez Rojas, MD.3 1 General Practitioner, Pediatrics Resident at the Abstract Universidad Pontificia Bolivariana in Medellín Colombia Cholestasis always indicates a pathological process that can result in chronic liver dysfunction, the necessity 2 Pediatric Gastro-hepatologist at Pablo Tobón Uribe of liver transplantation and even death. (1) Cholestasis is a process in which there is a decrease in biliary Hospital in Medellín, Colombia flow, histological evidence of deposition of bile pigments in hepatocytes and bile ducts, and an increase in the 3 Pediatrics Resident at the Universidad Militar Nueva Granada in Bogotá, Colombia serum concentrations of products excreted in bile. Cholestasis can occur at any age. It is caused by alteration of the formation of bile by the hepatocytes or by obstruction of the flow in the intrahepatic or extrahepatic biliary ......................................... tracts. Neonatal cholestasis occurs in the first (3) months of life with elevated serum levels of direct bilirubin, Received: 14-01-16 Accepted: 01-11-16 cholesterol and bile acids. (2) In our environment, the most frequent cause is idiopathic neonatal hepatitis, followed by infectious causes. Obstructive causes have the worst prognoses. Among them, the most common is biliary atresia in which progressive obliteration of the extra hepatic biliary tract, parenchymal damage and intrahepatic biliary tract cause cirrhosis and death before the patient reaches three years of age. (1-3) The prognosis improves with surgical management if it is performed within the first 45 to 60 days of life. -
Biliary Tract Cancer*
Biliary Tract Cancer* What is Biliary Tract Cancer*? Let us answer some of your questions. * Cholangiocarcinoma (bile duct cancer) * Gallbladder cancer * Ampullary cancer ESMO Patient Guide Series based on the ESMO Clinical Practice Guidelines esmo.org Biliary tract cancer Biliary tract cancer* An ESMO guide for patients Patient information based on ESMO Clinical Practice Guidelines This guide has been prepared to help you, as well as your friends, family and caregivers, better understand biliary tract cancer and its treatment. It contains information on the causes of the disease and how it is diagnosed, up-to- date guidance on the types of treatments that may be available and any possible side effects of treatment. The medical information described in this document is based on the ESMO Clinical Practice Guideline for biliary tract cancer, which is designed to help clinicians with the diagnosis and management of biliary tract cancer. All ESMO Clinical Practice Guidelines are prepared and reviewed by leading experts using evidence gained from the latest clinical trials, research and expert opinion. The information included in this guide is not intended as a replacement for your doctor’s advice. Your doctor knows your full medical history and will help guide you regarding the best treatment for you. *Cholangiocarcinoma (bile duct cancer), gallbladder cancer and ampullary cancer. Words highlighted in colour are defined in the glossary at the end of the document. This guide has been developed and reviewed by: Representatives of the European -
Pancreaticobiliary Ductal Union Gut: First Published As 10.1136/Gut.31.10.1144 on 1 October 1990
1144 Gut, 1990, 31, 1144-1149 Pancreaticobiliary ductal union Gut: first published as 10.1136/gut.31.10.1144 on 1 October 1990. Downloaded from S P Misra, M Dwivedi Abstract TABLE ii Length ofthe common channel in normalsubjects in The main pancreatic duct and the common bile various series duct open into the second part of the duo- Authors Mean (mm) Range (mm) denum alone or after joining as a common Misra et al4 4-7 1-018-4 channel. A common channel of >15 mm (an Kimura et al5 4.6 2-10 anomalous pancreaticobiliary duct) is associ- Dowdy et al6 4-4 1-12 ated with congenital cystic dilatation of the common bile duct and carcinoma of the gall bladder. Even a long common channel channel of <3 mm, and 18% had a common (38 mm) is associated with a higher frequency channel of >3 mm. The rhean length was not of carcinoma of the gall bladder. Gail stones mentioned.2 In a necropsy study of 35 infants, smaller than the common channel and a long Miyano et al' noted that the average length of the common channel predispose to gail stone common channel was 1 3 mm. induced acute pancreatitis. Separate openings Kimura et al,' using cineradiography during for the two ductal systems predisposes to ERCP, have shown contractile motility of the development of gall stones and alcohol ductal wall extending well beyond the common induced chronic pancreatitis. The role of channel, towards the liver. The mean (SD) ductal union has also been investigated in length of the contractile segment was 20 5 primary sclerosing cholangitis and biliary (4 6) mm (range 14-31 mm). -
Biliary Tract Obstruction in Chronic Pancreatitis
HPB, 2007; 9: 421Á428 REVIEW ARTICLE Biliary tract obstruction in chronic pancreatitis ABDUL A. ABDALLAH1, JAKE E. J. KRIGE2 & PHILIPPUS C. BORNMAN2 1Department of Surgery, Aga Khan University Hospital, Nairobi, Kenya and 2Department of Surgery, University of Cape Town Health Sciences Faculty, and Surgical Gastroenterology Unit, Groote Schuur Hospital, Observatory 7925, Cape Town, South Africa Abstract Bile duct strictures are a common complication in patients with advanced chronic pancreatitis and have a variable clinical presentation ranging from an incidental finding to overt jaundice and cholangitis. The diagnosis is mostly made during investigations for abdominal pain but jaundice may be the initial clinical presentation. The jaundice is typically transient but may be recurrent with a small risk of secondary biliary cirrhosis in longstanding cases. The management of a bile duct stricture is conservative in patients in whom it is an incidental finding as the risk of secondary biliary cirrhosis is negligible. Initial conservative treatment is advised in patients who present with jaundice as most will resolve once the acute on chronic attack has subsided. A surgical biliary drainage is indicated when there is persistent jaundice for more than one month or if complicated by secondary gallstones or cholangitis. The biliary drainage procedure of choice is a choledocho-jejunostomy which may be combined with a pancreaticojejunostomy in patients who have associated pain. Since many patients with chronic pancreatitis have an inflammatory mass in the head of the pancreas, a Frey procedure is indicated but a resection should be performed when there is concern about a malignancy. Temporary endoscopic stenting is reserved for cholangitis while an expandable metal stent may be indicated in patients with severe co-morbid disease. -
Primary Biliary Cholangitis: 2018 Practice Guidance from the American Association for the Study of Liver Diseases 1 2 3 4 5 Keith D
| PRACTICE GUIDANCE HEPATOLOGY, VOL. 0, NO. 0, 2018 Primary Biliary Cholangitis: 2018 Practice Guidance from the American Association for the Study of Liver Diseases 1 2 3 4 5 Keith D. Lindor, Christopher L. Bowlus, James Boyer, Cynthia Levy, and Marlyn Mayo Intended for use by health care providers, this guid- Preamble ance identifies preferred approaches to the diagnostic This American Association for the Study of and therapeutic aspects of care for patients with PBC. Liver Diseases (AASLD) 2018 Practice Guidance As with clinical practice guidelines, it provides gen- on Primary Biliary Cholangitis (PBC) is an update eral guidance to optimize the care of the majority of of the PBC guidelines published in 2009. The 2018 patients and should not replace clinical judgment for updated guidance on PBC includes updates on etiol- a unique patient. ogy and diagnosis, role of imaging, clinical manifesta- The major changes from the last guideline to this tions, and treatment of PBC since 2009. The AASLD guidance include information about obeticholic acid 2018 PBC Guidance provides a data-supported (OCA) and the adaptation of the guidance format. approach to screening, diagnosis, and clinical man- agement of patients with PBC. It differs from more recent AASLD practice guidelines, which are sup- Etiology of Primary Biliary ported by systematic reviews and a multidisciplinary panel of experts that rates the quality (level) of the Cholangitis evidence and the strength of each recommendation Primary Biliary Cholangitis (PBC) is considered using the Grading of Recommendations Assessment, an autoimmune disease because of its hallmark sero- Development, and Evaluation system. In contrast, this logic signature, antimitochondrial antibody (AMA), (1-4) guidance was developed by consensus of an expert and specific bile duct pathology. -
What Is the Normal Size of the Common Bile Duct?
DOI: https://doi.org/10.22516/25007440.136 Original articles What is the normal size of the common bile duct? Martín Alonso Gómez Zuleta, MD,1 Óscar Fernando Ruiz Morales, MD,2 William Otero Regino, MD.3 1 Internist and Gastroenterologist, Professor at the Abstract National University of Colombia. Gastroenterologist at National University of Colombia Hospital in Traditionally, the common bile duct (CBD) has been said to measure up to 6 mm in patients with gallbladders Bogotá, Colombia and up to 8 mm in cholecystectomized patients. However, these recommendations are based on very old 2 Internist and Gastroenterologist at Hospital Nacional studies performed with trans-abdominal ultrasound. Echoendoscopy has greater sensitivity and specificity for Universitario and Kennedy Hospital in Bogotá, Colombia evaluating the bile duct, but studies had not yet been done in our population to evaluate the normal size of 3 Internist and Gastroenterologist, Professor of the CBD by this method. Gastroenterology at the National University of Objective: The objective of this study was to evaluate the size of the CBD in patients with gallbladders and Colombia in Bogotá, Colombia patients without gallbladders. Materials and Methods: This is a prospective descriptive study of patients who underwent echoendoscopy ......................................... at the gastroenterology unit in the El Tunal hospital, Universidad Nacional de Colombia. Patients had been Received: 05-11-15 Accepted: 21-04-17 referred for diagnostic echoendoscopy to evaluate subepithelial lesions in the esophagus and/or stomach. Once the lesion had been evaluated and an echoendoscopic diagnosis had been established, the transducer was advanced to the second duodenal portion to perform bilio-pancreatic echoendoscopy.