Cholestasis and cholestatic syndromes.
OBJECTIVE
This review highlights recent advances in understanding the regulation of bile acid transport in cholestasis and in the pathogenesis, outcomes, epidemiology, and treatment of a variety of cholestatic liver diseases and their associated complications.


RESULTS
Highlights include additional understanding of the role of the nuclear receptors farsenoid X receptor, pregnane X receptor, and constitutive androstane receptor in bile acid homeostasis, new understanding of the pathogenesis of primary biliary cirrhosis, familial intrahepatic cholestasis, biliary atresia, and primary sclerosing cholangitis, and clinical trials of therapies for intrahepatic cholestasis of pregnancy, primary biliary cirrhosis, and primary sclerosing cholangitis.


CONCLUSIONS
Our understanding of the molecular mechanisms, epidemiology and pathogenesis of cholestasis continues to advance. These advances will hopefully lead to more effective therapies for specific cholestatic conditions.
Rutherford
Anna E
AE
Gastrointestinal Unit, Massachusetts General Hospital, Harvard Medical School, Boston, 02114, USA.
Pratt
Daniel S
DS
D000074020:ATP Binding Cassette Transporter, Subfamily B, Member 11; D018528:ATP-Binding Cassette Transporters / Q000235:genetics; D000818:Animals; D001647:Bile Acids and Salts / Q000378:metabolism; D001649:Bile Duct Diseases / Q000209:etiology / Q000503:physiopathology* / Q000628:therapy*; D001692:Biological Transport / Q000502:physiology; D005260:Female; D006801:Humans; D009154:Mutation; D010024:Osteoporosis / Q000188:drug therapy / Q000209:etiology; D011247:Pregnancy; D011248:Pregnancy Complications / Q000628:therapy; D011537:Pruritus / Q000188:drug therapy / Q000209:etiology
C093491:ABCB11 protein, human; D000074020:ATP Binding Cassette Transporter, Subfamily B, Member 11; D018528:ATP-Binding Cassette Transporters; D001647:Bile Acids and Salts