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Recurrence of Bile Salt Export Pump Deficiency after Liver Transplantation

2009; Massachusetts Medical Society; Volume: 361; Issue: 14 Linguagem: Inglês

10.1056/nejmoa0901075

1533-4406

Paloma Jara, Loreto Hierro, Pilar Martínez-Fernández, R Alvarez-Doforno, Francisca Yánez, Maria Carmen G. Diaz, C. Camarena, A. de la Vega, E. Frauca, Gema Muñoz‐Bartolo, Manuel López‐Santamaría, J Larrauri, Luis Álvarez,

Pharmacological Effects and Toxicity Studies

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.