Article ID | Journal | Published Year | Pages | File Type |
---|---|---|---|---|
4168658 | The Journal of Pediatrics | 2007 | 4 Pages |
Abstract
Fatal peripheral cholangiocarcinoma developed in 2 girls with progressive familial intrahepatic cholestasis, ABCB11 mutations, and absent bile salt export pump (BSEP) expression. BSEP deficiency may cause cholangiocarcinoma through bile-composition shifts or bile-acid damage within cells capable of hepatocytic/cholangiocytic differentiation. This observation suggests the need for hepatobiliary-malignancy surveillance and early consideration for liver transplantation.
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Authors
A.O. Scheimann, S.S. Strautnieks, A.S. Knisely, J.A. Byrne, R.J. Thompson, M.J. Finegold,