Ursodeoxycholic acid treatment of vanishing bile duct syndromes.
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.
Pusl
Thomas
T
Department of Medicine II, Klinikum Grosshadern, Marchioninistrasse 15, 81377 Munich, Germany.
Beuers
Ulrich
U
D017209:Apoptosis / Q000187:drug effects; D001647:Bile Acids and Salts / Q000494:pharmacology; D001649:Bile Duct Diseases / Q000150:complications / Q000188:drug therapy* / Q000473:pathology; D001653:Bile Ducts, Intrahepatic / Q000187:drug effects / Q000473:pathology*; D002756:Cholagogues and Choleretics / Q000378:metabolism / Q000493:pharmacokinetics / Q000494:pharmacology / Q000627:therapeutic use*; D002779:Cholestasis / Q000503:physiopathology; D006801:Humans; D008103:Liver Cirrhosis / Q000503:physiopathology; D017093:Liver Failure / Q000209:etiology / Q000473:pathology / Q000503:physiopathology; D013577:Syndrome; D014580:Ursodeoxycholic Acid / Q000378:metabolism / Q000493:pharmacokinetics / Q000494:pharmacology / Q000627:therapeutic use*
D001647:Bile Acids and Salts; D002756:Cholagogues and Choleretics; D014580:Ursodeoxycholic Acid