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Recurrence of bile salt export pump deficiency after liver transplantation. 2009
Paloma Jara, and
Loreto Hierro, and
Pilar Martínez-Fernández, and
Rita Alvarez-Doforno, and
Francisca Yánez, and
María C Diaz, and
Carmen Camarena, and
Angela De la Vega, and
Esteban Frauca, and
Gema Muñoz-Bartolo, and
Manuel López-Santamaría, and
Javier Larrauri, and
Luis Alvarez
Pediatric Liver Service, La Paz University Hospital, Madrid, Spain.
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.
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