Article
Recurrent low gamma-glutamyl transpeptidase cholestasis following liver transplantation for bile salt export pump (BSEP) disease (posttransplant recurrent BSEP disease).
Liver transplantation : official publication of the American Association for the Study of Liver Diseases and the International Liver Transplantation Society - 1 Jul 2010
Siebold Leah, Dick Andre A S, Thompson Richard, Maggiore Giuseppe, Jacquemin Emanuel, Jaffe Ronald, Strautnieks Sandra, Grammatikopoulos Tassos, Horslen Simon, Whitington Peter F, Shneider Benjamin L
Abstract excerpt
Bile salt export pump (BSEP) deficiency is a hereditary cholestatic syndrome that results from mutations in the ABCB11 (ATP-binding cassette B11) gene. Severely affected patients develop end-stage liver disease in the first decade of life. Liver transplantation has traditionally been thought of as curative for BSEP disease. We describe the clinical course of 6 patients who developed recurrent low gamma-glutamyl...
Topics
- ATP Binding Cassette Transporter, Subfamily B, Member 11
- ATP-Binding Cassette Transporters
- Child
- Child, Preschool
- Cholestasis
- Genotype
- Humans
- Infant