title: Therapeutic interventions in progressive familial intrahepatic cholestasis: experience from a tertiary care centre in north India.
abstract: Medical therapy has limited value in managing symptoms of progressive familial intrahepatic cholestasis (PFIC). Liver transplantation (LT) is the only definite therapy for progressive liver failure and intractable pruritis. In recent years, biliary diversion (BD) has also shown therapeutic promise. This study was designed to review the experience of management and outcome of seven PFIC patients. Two children each had type II and III and three had type I/II PFIC, respectively. Medical treatment was successful in only one. Decompensated cirrhosis had already set in four children. They underwent a living related LT. There was one post transplant mortality. Remaining all children had a normal graft function at a mean follow-up of 50 months. One patient of PFIC type I/II received internal and another of PFIC type II received external BD. Both patients were asymptomatic at follow-up of 19 and 23 months respectively. Nontransplant surgical options should be offered to noncirrhotic children with PFIC.
authors.affiliation: Pediatric Gastroenterology and Hepatology, Apollo Centre of Advanced Pediatrics, Indraprastha Apollo Hospital, New Delhi 110076, India.
authors.forename: Satvinder
authors.initials: S
authors.lastname: Kaur
authors.forename: Deepa
authors.initials: D
authors.lastname: Sharma
authors.forename: Nishant
authors.initials: N
authors.lastname: Wadhwa
authors.forename: Subash
authors.initials: S
authors.lastname: Gupta
authors.forename: Sujit Kumar
authors.initials: SK
authors.lastname: Chowdhary
authors.forename: Anupam
authors.initials: A
authors.lastname: Sibal
mesh_terms: D002648:Child; D002675:Child, Preschool; D002780:Cholestasis, Intrahepatic / Q000628:therapy*; D005260:Female; D006801:Humans; D007194:India; D007223:Infant; D008297:Male; D012189:Retrospective Studies