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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 https://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


Video abstract presented by Asudan Tugce Bozkurter Cil

Mithat Gunaydin,1 Asudan Tugce Bozkurter Cil2

1Avicenna Hospital, Department of Pediatric Surgery, Istanbul, Turkey; 2Medicana International Samsun Hospital, Department of Pediatric Surgery, Samsun, Turkey

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

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