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World J Gastroenterol. Jun 14, 2006; 12(22): 3487-3495
Published online Jun 14, 2006. doi: 10.3748/wjg.v12.i22.3487
Ursodeoxycholic acid treatment of vanishing bile duct syndromes
Thomas Pusl, Ulrich Beuers
Thomas Pusl, Ulrich Beuers, Department of Medicine II, Klinikum Grosshadern, University of Munich, Germany
Author contributions: All authors contributed equally to the work.
Correspondence to: Ulrich Beuers, MD, Department of Medicine II, Klinikum Grosshadern, Marchioninistrasse 15, 81377 Munich, Germany. beuers@med.uni-muenchen.de
Telephone: +49-89-70955272 Fax: +49-89-70955271
Received: January 31, 2006
Revised: March 5, 2006
Accepted: March 14, 2006
Published online: June 14, 2006
Abstract

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.

Keywords: Cholestasis; Primary biliary cirrhosis; Primary sclerosing cholangitis; Secretion; Signaling; Transport; Ursodeoxycholic acid; Vanishing bile duct syndrome