| Primary biliary cirrhosis: a 2010 update. | |
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MedLine Citation:
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PMID: 20347176 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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Primary biliary cirrhosis (PBC) is a chronic inflammatory autoimmune disease that mainly targets the cholangiocytes of the interlobular bile ducts in the liver. The condition primarily affects middle-aged women. Without treatment, PBC generally progresses to cirrhosis and eventually liver failure over a period of 10-20 years. PBC is a rare disease with prevalence of less than 1/2000. PBC is thought to result from a combination of multiple genetic factors and superimposed environmental triggers. The contribution of the genetic predisposition is evidenced by the familial clustering. Several risk factors, including exposure to infectious agents and chemical xenobiotics, have been suggested. Ursodeoxycholic acid (UDCA) is currently the only FDA-approved medical treatment for PBC. When administered at doses of 13-15 mg/kg/day, a majority of patients with PBC have a normal life expectancy without additional therapeutic measures. One out of three patients does not adequately respond to UDCA therapy and may need additional medical therapy and/or liver transplantation. This review summarises current knowledge on the epidemiology, ethiopathogenesis, clinical, and therapeutic aspects of PBC. |
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Authors:
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Raoul Poupon |
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Publication Detail:
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Type: Journal Article; Review Date: 2010-02-18 |
Journal Detail:
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Title: Journal of hepatology Volume: 52 ISSN: 0168-8278 ISO Abbreviation: J. Hepatol. Publication Date: 2010 May |
Date Detail:
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Created Date: 2010-05-03 Completed Date: 2010-08-10 Revised Date: - |
Medline Journal Info:
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Nlm Unique ID: 8503886 Medline TA: J Hepatol Country: England |
Other Details:
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Languages: eng Pagination: 745-58 Citation Subset: IM |
Copyright Information:
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Copyright (c) 2010 European Association for the Study of the Liver. Published by Elsevier B.V. All rights reserved. |
Affiliation:
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UPMC Univ Paris 06, France; INSERM, UMR_S 938, Paris, France. raoul.poupon@sat.aphp.fr |
Export Citation:
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| MeSH Terms | |
Descriptor/Qualifier:
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Diagnosis, Differential Environment Genetic Predisposition to Disease / genetics Hepatitis / immunology, pathology Humans Liver Cirrhosis, Biliary / epidemiology, genetics, immunology, physiopathology* Liver Diseases / epidemiology, genetics, immunology Lymphocytes / immunology, pathology Prevalence Prognosis Risk Factors |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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