Journal
WORLD JOURNAL OF GASTROENTEROLOGY
Volume 12, Issue 22, Pages 3487-3495Publisher
BAISHIDENG PUBLISHING GROUP INC
DOI: 10.3748/wjg.v12.i22.3487
Keywords
cholestasis; primary biliary cirrhosis; primary sclerosing cholangitis; secretion; signaling; transport; ursodeoxycholic acid; vanishing bile duct syndrome
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Vanishing bile duct syndromes (VBDS) are characterized by progressive loss of small intrahepatic ducts caused by a variety of different diseases leading to chronic cholestasis, cirrhosis, and premature death from liver failure. The majority of adult patients with VBDS suffer from primary biliary cirrhosis (PBC) and primary sclerosing cholangitis (PSC). Ursodeoxycholic acid (UDCA), a hydrophilic dihydroxy bile acid, is the only drug currently approved for the treatment of patients with PBC, and anticholestatic effects have been reported for several other cholestatic syndromes. Several potential mechanisms of action of UDCA have been proposed including stimulation of hepatobiliary secretion, inhibition of apoptosis and protection of cholangiocytes against toxic effects of hydrophobic bile acids. (C) 2006 The WJG Press. All rights reserved.
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