Successful pregnancy after ileal exclusion in progressive familial intrahepatic cholestasis type 2
Journal Title: Annals of Hepatology - Year 2015, Vol 14, Issue 4
Abstract
Progressive familial intrahepatic cholestasis type 2 (PFIC 2) results from mutations in ABCB11 gene coding bile salt export pump (BSEP). Medical treatment is usually unsuccessful and surgery intervention is necessary. Partial external biliary diversion (PEBD) is regarded as the first choice of surgical treatment. Ileal exclusion (IE) is an alternative operation if external stoma is not tolerated; however, a favorable outcome is uncertain. In chronic liver diseases pregnancy brings additional risk of deterioration of liver function and generally is not recommended. We present the first case report of successful pregnancy in a genetically confirmed PFIC 2 patient after surgical conversion from PEBD to IE.
Authors and Affiliations
Piotr Czubkowski, Irena Jankowska, Joanna Pawlowska
Keywords
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