Document Detail

Best practice guidance for the diagnosis and management of cystic fibrosis-associated liver disease.
MedLine Citation:
PMID:  21658639     Owner:  NLM     Status:  MEDLINE    
Approximately 5-10% of cystic fibrosis (CF) patients develop multilobular cirrhosis during the first decade of life. Most CF patients later develop signs of portal hypertension with complications, mainly variceal bleeding. Liver failure usually occurs later, after the paediatric age. Annual screening for liver disease is recommended to detect pre-symptomatic signs and initiate ursodeoxycholic acid therapy, which might halt disease progression. Liver disease should be considered if at least two of the following variables are present: abnormal physical examination, persistently abnormal liver function tests and pathological ultrasonography. If there is diagnostic doubt, a liver biopsy is indicated. All CF patients with liver disease need annual follow-up to evaluate the development of cirrhosis, portal hypertension or liver failure. Management should focus on nutrition, prevention of bleeding and variceal decompression. Deterioration of pulmonary function is an important consideration for liver transplantation, particularly in children with hepatic dysfunction or advanced portal hypertension.
Dominique Debray; Deirdre Kelly; Roderick Houwen; Birgitta Strandvik; Carla Colombo
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Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't; Review    
Journal Detail:
Title:  Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society     Volume:  10 Suppl 2     ISSN:  1873-5010     ISO Abbreviation:  J. Cyst. Fibros.     Publication Date:  2011 Jun 
Date Detail:
Created Date:  2011-06-10     Completed Date:  2011-09-02     Revised Date:  2012-02-21    
Medline Journal Info:
Nlm Unique ID:  101128966     Medline TA:  J Cyst Fibros     Country:  Netherlands    
Other Details:
Languages:  eng     Pagination:  S29-36     Citation Subset:  IM    
Copyright Information:
Copyright © 2011 European Cystic Fibrosis Society. Published by Elsevier B.V. All rights reserved.
Pediatric Hepatology, APHP-CHU Necker-Enfants Malades and INSERM, UMR-S 938, Centre de Recherche Saint-Antoine, Paris, France.
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MeSH Terms
Cystic Fibrosis / complications*
Esophageal and Gastric Varices / diagnosis,  etiology,  therapy
Liver Diseases* / diagnosis,  etiology,  therapy
Medicine / standards*
Practice Guidelines as Topic*
Comment In:
J Cyst Fibros. 2012 Jan;11(1):72-3; author reply 74-75   [PMID:  21862443 ]

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