PBC Primary Biliary Cholangitis Treatment and Management
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Evaluation of Abnormal Liver Chemistries
ACG Clinical Guideline: Evaluation of Abnormal Liver Chemistries Paul Y. Kwo, MD, FACG, FAASLD1, Stanley M. Cohen, MD, FACG, FAASLD2, and Joseph K. Lim, MD, FACG, FAASLD3 1Division of Gastroenterology/Hepatology, Department of Medicine, Stanford University School of Medicine, Palo Alto, California, USA; 2Digestive Health Institute, University Hospitals Cleveland Medical Center and Division of Gastroenterology and Liver Disease, Department of Medicine, Case Western Reserve University School of Medicine, Cleveland, Ohio, USA; 3Yale Viral Hepatitis Program, Yale University School of Medicine, New Haven, Connecticut, USA. Am J Gastroenterol 2017; 112:18–35; doi:10.1038/ajg.2016.517; published online 20 December 2016 Abstract Clinicians are required to assess abnormal liver chemistries on a daily basis. The most common liver chemistries ordered are serum alanine aminotransferase (ALT), aspartate aminotransferase (AST), alkaline phosphatase and bilirubin. These tests should be termed liver chemistries or liver tests. Hepatocellular injury is defined as disproportionate elevation of AST and ALT levels compared with alkaline phosphatase levels. Cholestatic injury is defined as disproportionate elevation of alkaline phosphatase level as compared with AST and ALT levels. The majority of bilirubin circulates as unconjugated bilirubin and an elevated conjugated bilirubin implies hepatocellular disease or cholestasis. Multiple studies have demonstrated that the presence of an elevated ALT has been associated with increased liver-related mortality. A true healthy normal ALT level ranges from 29 to 33 IU/l for males, 19 to 25 IU/l for females and levels above this should be assessed. The degree of elevation of ALT and or AST in the clinical setting helps guide the evaluation. -
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. -
Non-Hepatic Hyperammonaemia: an Important, Potentially Reversible Cause of Encephalopathy
Postgrad Med J 2001;77:717–722 717 Postgrad Med J: first published as 10.1136/pmj.77.913.717 on 1 November 2001. Downloaded from CASE REPORTS Non-hepatic hyperammonaemia: an important, potentially reversible cause of encephalopathy N D Hawkes, G A O Thomas, A Jurewicz, O M Williams, C E M Hillier, I N F McQueen, G Shortland Abstract Case reports The clinical syndrome of encephalopathy CASE 1 is most often encountered in the context of A 20 year old man was admitted to a local hos- decompensated liver disease and the diag- pital with two days of inappropriate behaviour, nosis is usually clear cut. Non-hepatic clumsiness, drowsiness, memory loss, slurred causes of encephalopathy are rarer and speech, and abdominal discomfort. Since the tend to present to a wide range of medical age of 2 years he had suVered recurrent rectal specialties with variable and episodic bleeding and investigation had revealed haem- symptoms. Delay can result in the devel- orrhoids. Bleeding from his rectum had contin- opment of potentially life threatening ued over the years but had been worse recently. complications, such as seizures and coma. On examination he was confused. His Early recognition is vital. A history of Glasgow coma scale score (GCS) was 15/15. similar episodes or clinical risk factors Neurological and general examination was nor- and early assessment of blood ammonia mal, with no stigmata of chronic liver disease. levels help establish the diagnosis. In Investigations showed a leucocytosis (leuco- × 9 addition to adequate supportive care, cyte count 22 10 /l) and serum bilirubin level investigation of the underlying cause of of 32 µmol/l. -
Venous Complications of Pancreatitis: a Review Yashant Aswani, Priya Hira Department of Radiology, Seth GS Medical College and KEM Hospital, Mumbai, Maharashtra INDIA
JOP. J Pancreas (Online) 2015 Jan 31; 16(1):20-24 REVIEW ARTICLE Venous Complications of Pancreatitis: A Review Yashant Aswani, Priya Hira Department of Radiology, Seth GS Medical College and KEM Hospital, Mumbai, Maharashtra INDIA ABSTRACT Pancreatitis is notorious to cause vascular complications. While arterial complications include pseudoaneurysm formation with a propensity to bleed, unusual venous complications associated with pancreatitis have, however, been described. In this article, we review multitudinous venous complications in thevenous setting complications of pancreatitis can andbe quite propose myriad. a system Venous to involvementclassify pancreatitis in pancreatitis associated often venous presents complications. with thrombosis. From time to time case reports and series of INTRODUCTION THROMBOTIC COMPLICATIONS IN PANCREATITIS Venous thrombosis is the most common complication of pancreatitis mediators and digestive enzymes. Consequently, pancreatitis associated complicationsPancreatitis is cana systemic be myriad disease with vascularowing to complications release of inflammatory being a well known but infrequent phenomenon. These vascular complications affecting venous system. A surge in procoagulant inflammatory mediators, are seen in 25% patients suffering from pancreatitis and entail chronicstasis, vesselpancreatitis spasm, (CP) mass includes effects intimal from injury the surroundingdue to repeated inflamed acute pancreas causes thrombosis in acute pancreatitis [2] whereas etiology in of peripancreatic arteries. Venous complications are less commonly significant morbidity and mortality [1]. There is predominant affliction tiesinflammation, with pancreas chronic results inflammation in splenic veinwith involvement fibrosis, compressive in majority effectsof the of a pseudocyst or an enlarged inflamed pancreas [3]. Close anatomic complicationsreported and associatedare often withconfined pancreatitis to thrombosis have, however, of the been vein. described. Isolated 22% (Agrawal et al.) and 5.6% (Bernades et al.), respectively. -
Review Article Pruritus in Systemic Diseases: a Review of Etiological Factors and New Treatment Modalities
Hindawi Publishing Corporation e Scientific World Journal Volume 2015, Article ID 803752, 8 pages http://dx.doi.org/10.1155/2015/803752 Review Article Pruritus in Systemic Diseases: A Review of Etiological Factors and New Treatment Modalities Nagihan Tarikci, Emek Kocatürk, Fule Güngör, IlteriG OLuz Topal, Pelin Ülkümen Can, and Ralfi Singer Department of Dermatology, Okmeydanı Training and Research Hospital, 34384 Istanbul, Turkey Correspondence should be addressed to Emek Kocaturk;¨ [email protected] Received 20 February 2015; Revised 11 June 2015; Accepted 16 June 2015 Academic Editor: Uwe Wollina Copyright © 2015 Nagihan Tarikci et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. Pruritus is the most frequently described symptom in dermatology and can significantly impair the patient’s quality of life. In 10–50% of adults with persistent pruritus, it can be an important dermatologic clue for the presence of a significant underlying systemic disease such as renal insufficiency, cholestasis, hematologic disorder, or malignancy (Etter and Myers, 2002; Zirwas and Seraly, 2001). This review describes the presence of pruritus in different systemic diseases. It is quite important to discover the cause of pruritus for providing relief for the patients experiencing substantial morbidity caused by this condition. 1. Pruritus Endocrinal Disorders. Thyroid diseases, diabetes mellitus. Pruritus is a topic that has caused a great deal of controversy Paraneoplastic Diseases. Lymphomas and solid organ tumors. because it is difficult to characterize and define. Various indirect definitions proposed include a sensation which provokes the desire to scratch or an uneasy sensation of 2. -
Acute Liver Failure J G O’Grady
148 Postgrad Med J: first published as 10.1136/pgmj.2004.026005 on 4 March 2005. Downloaded from REVIEW Acute liver failure J G O’Grady ............................................................................................................................... Postgrad Med J 2005;81:148–154. doi: 10.1136/pgmj.2004.026005 Acute liver failure is a complex multisystemic illness that account for most cases, but a significant number of patients have no definable cause and are evolves quickly after a catastrophic insult to the liver classified as seronegative or of being of indeter- leading to the development of encephalopathy. The minate aetiology. Paracetamol is the commonest underlying aetiology and the pace of progression strongly cause in the UK and USA.2 Idiosyncratic reac- tions comprise another important group. influence the clinical course. The commonest causes are paracetamol, idiosyncratic drug reactions, hepatitis B, and Viral seronegative hepatitis. The optimal care is multidisciplinary ALF is an uncommon complication of viral and up to half of the cases receive liver transplants, with hepatitis, occurring in 0.2%–4% of cases depend- ing on the underlying aetiology.3 The risk is survival rates around 75%–90%. Artificial liver support lowest with hepatitis A, but it increases with the devices remain unproven in efficacy in acute liver failure. age at time of exposure. Hepatitis B can be associated with ALF through a number of ........................................................................... scenarios (table 2). The commonest are de novo infection and spontaneous surges in viral repli- cation, while the incidence of the delta virus cute liver failure (ALF) is a complex infection seems to be decreasing rapidly. multisystemic illness that evolves after a Vaccination should reduce the incidence of Acatastrophic insult to the liver manifesting hepatitis A and B, while antiviral drugs should in the development of a coagulopathy and ameliorate replication of hepatitis B. -
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. -
Report from the Inaugural Australian Pruritus Symposium, Sydney, Australia, August 10, 2013
Acta Derm Venereol 2014; 94: 123 LETTER TO THE EDITOR Report from the Inaugural Australian Pruritus Symposium, Sydney, Australia, August 10, 2013 Frank Brennan1 and Dedee F. Murrell2* 1Palliative Medicine, Calvary Hospital, 91 Rocky Point Road, and 2Department of Dermatology, St George Hospital, University of New South Wales, Gray St, Kogarah, Sydney, NSW 2217 Australia. *E-mail: [email protected] Accepted Aug 28, 2013; Epub ahead of print Oct 24, 2013 Sir, on the mechanisms and management of opioid-induced The inaugural Australian symposium on pruritus was itch. Frank Brennan spoke on uraemic pruritus, Paul Gray, convened at St George Hospital, Sydney on August 10, a Pain Specialist with a particular interest in burns spoke 2013. The co-conveners were Professor Dedee Murrell, on the phenomenon of post-burns pruritus and Craig Le- Executive Vice President of the International Society of wis, Medical Oncologist surveyed the symptom of itch Dermatology (ISD) and Dr Frank Brennan, Palliative and its management in cancer medicine. Medicine Physician. The impetus behind the symposium A feature of the day was an interview with a patient was the recognition of two facts. Firstly, the significant in front of the symposium participants. The patient developments in the understanding of the pathophysio- presented with a challenging combination of pruritus logy of pruritus in recent years and, secondly, the paucity secondary to a life-long history of atopy and, in later of education and understanding by colleagues across years, uraemic pruritus. Connie Katelaris surveyed the multiple disciplines of those developments. Given that history and immunological results of the patient and the symptom of pruritus manifests in many diseases the made clinical recommendations. -
European Guideline Chronic Pruritus Final Version
EDF-Guidelines for Chronic Pruritus In cooperation with the European Academy of Dermatology and Venereology (EADV) and the Union Européenne des Médecins Spécialistes (UEMS) E Weisshaar1, JC Szepietowski2, U Darsow3, L Misery4, J Wallengren5, T Mettang6, U Gieler7, T Lotti8, J Lambert9, P Maisel10, M Streit11, M Greaves12, A Carmichael13, E Tschachler14, J Ring3, S Ständer15 University Hospital Heidelberg, Clinical Social Medicine, Environmental and Occupational Dermatology, Germany1, Department of Dermatology, Venereology and Allergology, Wroclaw Medical University, Poland2, Department of Dermatology and Allergy Biederstein, Technical University Munich, Germany3, Department of Dermatology, University Hospital Brest, France4, Department of Dermatology, Lund University, Sweden5, German Clinic for Diagnostics, Nephrology, Wiesbaden, Germany6, Department of Psychosomatic Dermatology, Clinic for Psychosomatic Medicine, University of Giessen, Germany7, Department of Dermatology, University of Florence, Italy8, Department of Dermatology, University of Antwerpen, Belgium9, Department of General Medicine, University Hospital Muenster, Germany10, Department of Dermatology, Kantonsspital Aarau, Switzerland11, Department of Dermatology, St. Thomas Hospital Lambeth, London, UK12, Department of Dermatology, James Cook University Hospital Middlesbrough, UK13, Department of Dermatology, Medical University Vienna, Austria14, Department of Dermatology, Competence Center for Pruritus, University Hospital Muenster, Germany15 Corresponding author: Elke Weisshaar -
Picquestion of the Week:3/09/09
McKechnie Field - Spring Training Home of the Pirates PIC QUESTION OF THE WEEK: 3/09/09 Q: Why is rifaximin used in patients with pouchitis? A: Pouchitis is an inflammation of the internal pouch fashioned from small intestinal tissue during ileal pouch anal anastamosis (IPAA). This surgical procedure bypasses the large intestine and may be employed in patients with ulcerative colitis or Crohn’s disease. A temporary ileostomy is placed to allow the pouch to heal without risk of infection. IPAA is considered preferable to an ostomy for patients suffering from inflammatory bowel diseases refractory to medical treatment. The pouch serves as a collection device for waste, but permits the patient to experience regular bowel movements. It is typical for patients with an internal pouch to experience more frequent (average 6 per day) and watery bowel movements. Pouchitis is the most common complication of IPAA and widely regarded as an idiopathic disease; however, colonization by fecal bacteria may be a contributing factor. The condition occurs most commonly in the first six months following reversal of the ileostomy. Although its frequency decreases after six months, nearly 50% of patients with an IPAA will eventually experience pouchitis. Presenting symptoms include diarrhea, increased frequency of bowel movements, bleeding, abdominal pain, and fever. It can result in dehydration and, in severe cases, hospitalization. Treatment of acute pouchitis generally consists of a two-week course of antibiotic therapy with metronidazole or ciprofloxacin. Approximately 10% of patients do not respond to initial treatment and develop chronic (> 4 weeks) pouchitis. A small number of clinical trials support the potential use of rifaximin for the treatment of refractory or recurrent pouchitis. -
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. -
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.