Journal of Indian Association of Pediatric Surgeons (Jan 2019)
Two cases of progressive familial intrahepatic cholestasis type 2: Role of surgery with brief review of literature
Abstract
Progressive familial intrahepatic cholestasis (PFIC) is a rare bile acid transporter defect and autosomal recessive disorder with type 2 being the most common type. Partial internal or external biliary diversion delays its progression to end-stage liver disease. Here, we discuss two cases of type 2 PFIC.
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