Document Detail

A two-year prospective study of the effect of ursodeoxycholic acid on urinary bile acid excretion and liver morphology in cystic fibrosis-associated liver disease.
MedLine Citation:
PMID:  9425933     Owner:  NLM     Status:  MEDLINE    
The efficacy of 2 years of treatment with ursodeoxycholic acid (UDCA) in cystic fibrosis (CF)-associated liver disease was evaluated by liver biopsies and liver function tests in 10 patients aged 8 to 28 years. The metabolism of UDCA was investigated by analysis of urinary bile acids with fast atom bombardment mass spectrometry (FABMS) and gas-liquid chromatography-mass spectrometry. Eight patients responded with normalization of liver function tests (LFT) and all with decreased serum levels of immunoglobulin G (IgG). Blind evaluation of liver biopsies indicated improved liver morphology with less inflammation and/or bile duct proliferation than before treatment with UDCA in 7 patients. Only 1 patient had signs of progression of clinical liver disease. The proportion of UDCA and isoUDCA in urine varied, but increased during treatment from a mean (median) of approximately 4% (3%) to 40% (40%) of total bile acids. The increase was not related to LFT. The secondary bile acids, such as lithocholic acid (LCA) and deoxycholic acid (DCA), did not increase significantly. The excretion pattern of glycosidic conjugates of UDCA and its metabolites was similar to that found in healthy individuals, UDCA and isoUDCA being mainly excreted in conjugation with N-acetylglucosamine. This study shows that UDCA modulates inflammation in CF-associated liver disease and indicates improvement of liver morphology during 2 years of treatment.
A Lindblad; H Glaumann; B Strandvik
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Publication Detail:
Type:  Clinical Trial; Journal Article; Research Support, Non-U.S. Gov't    
Journal Detail:
Title:  Hepatology (Baltimore, Md.)     Volume:  27     ISSN:  0270-9139     ISO Abbreviation:  Hepatology     Publication Date:  1998 Jan 
Date Detail:
Created Date:  1998-02-02     Completed Date:  1998-02-02     Revised Date:  2006-11-15    
Medline Journal Info:
Nlm Unique ID:  8302946     Medline TA:  Hepatology     Country:  UNITED STATES    
Other Details:
Languages:  eng     Pagination:  166-74     Citation Subset:  IM    
Department of Pediatrics, Sahlgrenska University Hospital, Göteborg University, Sweden.
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MeSH Terms
Bile Acids and Salts / urine*
Cystic Fibrosis / complications*
Gas Chromatography-Mass Spectrometry
Liver / drug effects*,  pathology,  physiopathology
Liver Diseases / drug therapy*,  etiology*,  pathology
Liver Function Tests
Prospective Studies
Spectrometry, Mass, Fast Atom Bombardment
Treatment Outcome
Ursodeoxycholic Acid / therapeutic use*
Reg. No./Substance:
0/Bile Acids and Salts; 128-13-2/Ursodeoxycholic Acid

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine

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