Recurrence of bile salt export pump deficiency after liver transplantation. The New England journal of medicine [N Engl J Med] Journal article

Title	Recurrence of bile salt export pump deficiency after liver transplantation.
Author(s)	Jara P, Hierro L, Martínez-Fernández P, Alvarez-Doforno R, Yánez F, Diaz MC, Camarena C, De la Vega A, Frauca E, Muñoz-Bartolo G, López-Santamaría M, Larrauri J, Alvarez L
Institution	Pediatric Liver Service, La Paz University Hospital, Madrid, Spain.
Source	N Engl J Med 2009 Oct 1; 361(14):1359-67.
MeSH	ATP-Binding Cassette Transporters Animals Autoantibodies Bile Acids and Salts Child, Preschool Cholestasis Female Humans Immunosuppression Jaundice Liver Liver Transplantation Male Multidrug Resistance-Associated Proteins Phenotype Pruritus Rats Rats, Sprague-Dawley Remission Induction Sequence Analysis, DNA
Abstract	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 regimen. The phenotypic recurrence of the disease correlated with the presence of circulating high-titer antibodies against BSEP that inhibit transport by BSEP in vitro. When administered to rats, these antibodies targeted the bile canaliculi and impaired bile acid secretion.
Language	eng
Pub Type(s)	Journal Article Research Support, Non-U.S. Gov't
PubMed ID	19797282

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