Mehl A, Bohorquez H, Serrano MS, Galliano G, Reichman TW. Liver transplantation and the management of progressive familial intrahepatic cholestasis in children. World J Transplant 2016; 6(2): 278-290 [PMID: 27358773 DOI: 10.5500/wjt.v6.i2.278]
Corresponding Author of This Article
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
Research Domain of This Article
Transplantation
Article-Type of This Article
Review
Open-Access Policy of This Article
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/
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
Abstract
Progressive familial intrahepatic cholestasis (PFIC) is a constellation of inherited disorders that result in the impairment of bile flow through the liver that predominantly affects children. The accumulation of bile results in progressive liver damage, and if left untreated leads to end stage liver disease and death. Patients often present with worsening jaundice and pruritis within the first few years of life. Many of these patients will progress to end stage liver disease and require liver transplantation. The role and timing of liver transplantation still remains debated especially in the management of PFIC1. In those patients who are appropriately selected, liver transplantation offers an excellent survival benefit. Appropriate timing and selection of patients for liver transplantation will be discussed, and the short and long term management of patients post liver transplantation will also be described.
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.
