Recurrence of Bile Salt Export Pump Deficiency after Liver Transplantation  

Oleh:

Jara, Paloma ; Hierro, Loreto ; Martinez-Fernandez, Pilar ; Alvarez-Doforno, Rita ; Yanez, Francisca

Jenis: Article from Journal - ilmiah internasional
Dalam koleksi: The New England Journal of Medicine (keterangan: ada di Proquest) vol. 361 no. 14 (Oct. 2009), page 1359-1367 .
Topik: Bile salt; liver transplantation

Ketersediaan

Perpustakaan FK Nomor Panggil: N08.K.2009.05 Non-tandon: 1 (dapat dipinjam: 0) Tandon: tidak ada

Lihat Detail Induk

Isi artikel

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.

Opini Anda

Klik untuk menuliskan opini Anda tentang koleksi ini!

Kembali