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Progressive familial intrahepatic cholestasis: diagnosis, management, and treatment
Authors Gunaydin M, Bozkurter Cil AT
Received 24 November 2017
Accepted for publication 6 July 2018
Published 10 September 2018 Volume 2018:10 Pages 95—104
DOI http://doi.org/10.2147/HMER.S137209
Checked for plagiarism Yes
Review by Single-blind
Peer reviewers approved by Dr Justinn Cochran
Peer reviewer comments 4
Editor who approved publication: Dr Gerry Lake-Bakaar
Abstract: Progressive familial intrahepatic cholestasis (PFIC) is a group of autosomal recessive cholestatic liver diseases which are subgrouped according to the genetic defect, clinical presentation, laboratory findings and liver histology. Progressive liver fibrosis, cirrhosis, and end stage liver disease (ESLD) may eventually develop. PFIC was first described in Amish descendants of Jacob Byler, therefore it was originally called Byler disease. But it can be seen anywhere on the globe. This review summarizes the main features of the subtypes of the disease and discusses the current available diagnosis, conservative and surgical therapeutic options.
Keywords: intrahepatic cholestasis, jaundice, biliary diversion
摘要视频链接：Progressive familial intrahepatic cholestasis
