| Hepatobiliary disease in patients with cystic fibrosis. | |
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MedLine Citation:
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PMID: 19381084 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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PURPOSE OF REVIEW: This review explores the recent advances in knowledge regarding hepatobiliary disease in patients with cystic fibrosis. RECENT FINDINGS: Hepatobiliary abnormalities associated with cystic fibrosis are varied in nature and range from defects attributable to the underlying genetic defect to those related to systemic disease and malnutrition. Novel research into the underlying pathogenesis of cystic fibrosis liver disease and the primary role of cystic fibrosis transmembrane conductance regulator in biliary secretory epithelium is presented. This work has been fostered by the development of new animal models of cystic fibrosis transmembrane conductance regulator dysfunction. Of the wide range of hepatobiliary complications associated with cystic fibrosis the most clinically relevant problem is progression of focal biliary cirrhosis to multilobular cirrhosis with its attendant complications of portal hypertension and potentially end-stage liver disease. However, recent studies suggest that liver transplantation may not improve survival in patients with cystic fibrosis and significant portal hypertension. SUMMARY: Hepatobiliary disease is a common finding in patients with cystic fibrosis; the pathogenesis is multifactorial in nature. As new therapeutic strategies emerge, life expectancy will continue to increase as will the impact of liver disease on quality of life and survival of patients with cystic fibrosis. This review will discuss novel insights into pathogenesis as well as diagnostic and management options. |
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Authors:
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Katie Moyer; William Balistreri |
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Publication Detail:
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Type: Journal Article; Review |
Journal Detail:
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Title: Current opinion in gastroenterology Volume: 25 ISSN: 1531-7056 ISO Abbreviation: Curr. Opin. Gastroenterol. Publication Date: 2009 May |
Date Detail:
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Created Date: 2009-04-23 Completed Date: 2009-09-03 Revised Date: - |
Medline Journal Info:
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Nlm Unique ID: 8506887 Medline TA: Curr Opin Gastroenterol Country: United States |
Other Details:
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Languages: eng Pagination: 272-8 Citation Subset: IM |
Affiliation:
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Liver Care Center, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio 45229-3039, USA. |
Export Citation:
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| MeSH Terms | |
Descriptor/Qualifier:
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Animals Bile Duct Diseases / etiology* Cystic Fibrosis / complications* Humans Liver Diseases / etiology* Risk Factors |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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