Review
Copyright ©The Author(s) 2016. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Transplant. Jun 24, 2016; 6(2): 278-290
Published online Jun 24, 2016. doi: 10.5500/wjt.v6.i2.278
Liver transplantation and the management of progressive familial intrahepatic cholestasis in children
Ashley Mehl, Humberto Bohorquez, Maria-Stella Serrano, Gretchen Galliano, Trevor W Reichman
Ashley Mehl, University of Queensland-Ochsner Clinical School of Medicine, Jefferson, LA 70118, United States
Humberto Bohorquez, Trevor W Reichman, Multi-Organ Transplant Institute, Ochsner Medical Center, New Orleans, LA 70118, United States
Maria-Stella Serrano, Section of Pediatric Gastroenterology, Hepatology, and Nutrition, Department of Pediatrics, Ochsner Medical Center, New Orleans, LA 70118, United States
Gretchen Galliano, Department of Pathology, Ochsner Medical Center, New Orleans, LA 70118, United States
Author contributions: All authors contributed to this manuscript.
Conflict-of-interest statement: The authors have indicated they have no potential conflicts of interest to disclose.
Open-Access: This article is an open-access article which was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/
Correspondence to: Trevor W Reichman, MD, PhD, FACS, Multi-Organ Transplant Institute, Ochsner Medical Center, 1514 Jefferson Hwy, New Orleans, LA 70118, United States. treichman@ochsner.org
Telephone: +1-504-8423925 Fax: +1-504-8425746
Received: December 14, 2015
Peer-review started: December 18, 2015
First decision: January 18, 2016
Revised: February 24, 2016
Accepted: March 9, 2016
Article in press: March 14 2016
Published online: June 24, 2016
Core Tip

Core tip: Progressive familial intrahepatic cholestasis is a rare disorder that predominantly affects young children. If left untreated, children develop debilitating cholestasis and eventually progress to liver failure. Liver transplantation is curative of symptoms related to liver disease but in some cases worsens the extrahepatic symptoms. A multidisciplinary approach is critical to obtaining good long-term outcomes.