Therapeutic interventions in progressive familial intrahepatic cholestasis: experience from a tertiary care centre in north India.

摘要: Medical therapy has limited value in managing symptoms of progressive familial intrahepatic cholestasis (PFIC). Liver transplantation (LT) is the only definite for liver failure and intractable pruritis. In recent years, biliary diversion (BD) also shown therapeutic promise. This study was designed to review experience management outcome seven PFIC patients. Two children each had type II III three I/II PFIC, respectively. treatment successful one. Decompensated cirrhosis already set four children. They underwent a living related LT. There one post transplant mortality. Remaining all normal graft function at mean follow-up 50 months. One patient received internal another external BD. Both patients were asymptomatic 19 23 months Nontransplant surgical options should be offered noncirrhotic with PFIC.