Article
Recurrence of bile salt export pump deficiency after liver transplantation.
The New England journal of medicine - 1 Oct 2009
Jara Paloma, Hierro Loreto, Martínez-Fernández Pilar, Alvarez-Doforno Rita, Yánez Francisca, Diaz María C, Camarena Carmen, De la Vega Angela, Frauca Esteban, Muñoz-Bartolo Gema, López-Santamaría Manuel, Larrauri Javier, Alvarez Luis
Abstract excerpt
Severe bile salt export pump (BSEP) deficiency is a hereditary cholestatic condition that starts in infancy and leads to end-stage liver disease. Three children who underwent orthotopic liver transplantation for severe BSEP deficiency had post-transplantation episodes of cholestatic dysfunction that mimicked the original disease. Remission of all episodes was achieved by intensifying the immunosuppressive...
Topics
- ATP Binding Cassette Transporter, Subfamily B, Member 11
- ATP-Binding Cassette Transporters
- Animals
- Autoantibodies
- Bile Acids and Salts
- Child, Preschool
- Cholestasis
- Female
- Humans