Article
Hepatocellular carcinoma in ten children under five years of age with bile salt export pump deficiency.
Hepatology (Baltimore, Md.) - 1 Aug 2006
Knisely A S, Strautnieks Sandra S, Meier Yvonne, Stieger Bruno, Byrne Jane A, Portmann Bernard C, Bull Laura N, Pawlikowska Ludmila, Bilezikçi Banu, Ozçay Figen, László Aranka, Tiszlavicz László, Moore Lynette, Raftos Jeremy, Arnell Henrik, Fischler Björn, Németh Antal, Papadogiannakis Nikos, Cielecka-Kuszyk Joanna, Jankowska Irena, Pawłowska Joanna, Melín-Aldana Hector, Emerick Karan M, Whitington Peter F, Mieli-Vergani Giorgina, Thompson Richard J
Abstract excerpt
Hepatocellular carcinoma (HCC) is rare in young children. We attempted to see if immunohistochemical and mutational-analysis studies could demonstrate that deficiency of the canalicular bile acid transporter bile salt export pump (BSEP) and mutation in ABCB11, encoding BSEP, underlay progressive familial intrahepatic cholestasis (PFIC)--or "neonatal hepatitis" suggesting PFIC--that was associated with HCC in...
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