Outcomes following partial external biliary diversion in patients with progressive familial intrahepatic cholestasis.
Sign Up to like & getrecommendations! Published in 2017 at "Journal of pediatric surgery"
DOI: 10.1016/j.jpedsurg.2016.11.021
Abstract: BACKGROUND/PURPOSE PFIC is a family of bile acid (BA) transport disorders that may result in serious liver disease requiring transplantation. We reviewed our experience with PEBD as a method to improve liver function and avoid… read more here.
Keywords: pfic patients; partial external; pfic pfic; outcomes following ... See more keywords
Odevixibat and partial external biliary diversion showed equal improvement of cholestasis in a patient with progressive familial intrahepatic cholestasis
Sign Up to like & getrecommendations! Published in 2020 at "BMJ Case Reports"
DOI: 10.1136/bcr-2019-234185
Abstract: Untreated progressive familial intrahepatic cholestasis (PFIC) type 2, or bile salt exporter protein deficiency, frequently leads to severe pruritus, impaired growth and progressive liver fibrosis with risk of organ failure. We describe a 15-month-old male… read more here.
Keywords: partial external; familial intrahepatic; progressive familial; intrahepatic cholestasis ... See more keywords