Open AccessTwo cases of progressive familial intrahepatic cholestasis type 2: Role of surgery with brief review of literature
Author(s) -
Hinglaj Saha,
Tapanjyoti Ghosh,
Soumali Biswas,
Prafulla Kumar Mishra,
Kalyani Saha Basu,
Uttara Chatterjee
Publication year - 2019
Publication title -
journal of indian association of pediatric surgeons
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.306
H-Index - 17
eISSN - 1998-3891
pISSN - 0971-9261
DOI - 10.4103/jiaps.jiaps_235_17
Subject(s) - progressive familial intrahepatic cholestasis , medicine , cholestasis , gastroenterology , bile acid , surgery , liver transplantation , transplantation
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.