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

  • Hinglaj Saha,
  • Ghosh Tapanjyoti,
  • Somak Biswas,
  • Prafulla Kumar Mishra,
  • Kalyani Saha Basu,
  • Uttara Chatterjee

DOI
https://doi.org/10.4103/jiaps.JIAPS_235_17
Journal volume & issue
Vol. 24, no. 1
pp. 75 – 77

Abstract

Read online

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.

Keywords