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©The Author(s) 2017. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Gastroenterol. Feb 7, 2017; 23(5): 763-775
Published online Feb 7, 2017. doi: 10.3748/wjg.v23.i5.763
Current and future therapies for inherited cholestatic liver diseases
Stan FJ van de Graaf, Roderick HJ Houwen, Wendy L van der Woerd
Wendy L van der Woerd, Stan FJ van de Graaf, Tytgat Institute for Liver and Intestinal Research, Academic Medical Center, 1105 BK Amsterdam, The Netherlands
Wendy L van der Woerd, Roderick HJ Houwen, Department of Pediatric Gastroenterology, Wilhelmina Children’s Hospital, University Medical Centre Utrecht, 3584 EA Utrecht, The Netherlands
Stan FJ van de Graaf, Department of Gastroenterology and Hepatology, Academic Medical Center, University of Amsterdam, 1105 BK Amsterdam, The Netherlands
Author contributions: van der Woerd WL wrote the manuscript; van de Graaf SFJ created the figures; Houwen RHJ and van de Graaf SFJ reviewed and revised the manuscript; all authors read and approved the final version of the manuscript.
Conflict-of-interest statement: Authors declare no conflict of interest for this article.
Correspondence to: Wendy L van der Woerd, MD, PhD, Department of Pediatric Gastroenterology, Wilhelmina Children’s Hospital, University Medical Centre Utrecht, Lundlaan 6, 3584 EA Utrecht, The Netherlands. wwoerd@umcutrecht.nl
Telephone: +31-88-7555555 Fax: +31-88-7555312
Received: July 6, 2016
Peer-review started: July 9, 2016
First decision: September 12, 2016
Revised: November 16, 2016
Accepted: January 11, 2017
Article in press: January 11, 2017
Published online: February 7, 2017
Processing time: 199 Days and 16.2 Hours
Core Tip

Core tip: Familial intrahepatic cholestasis (FIC) is a group of autosomal recessive liver diseases characterized by intrahepatic cholestasis. Phenotypes vary from only episodic disease to progressive FIC. Current therapeutic options are often insufficient to prevent progression of the disease. This review will discuss the current therapeutic regimen as well as the development of novel therapeutic strategies, focusing on surgical and pharmacological biliary diversion, hepatocyte transplantation and mutation-specific therapy.