Brief Article
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World J Hepatol. Mar 27, 2013; 5(3): 127-132
Published online Mar 27, 2013. doi: 10.4254/wjh.v5.i3.127
Liver transplantation in Wilson’s disease: Single center experience from Saudi Arabia
Musthafa Chalikandy Peedikayil, Hamad Ibrahim Al Ashgar, Abdullah Al Mousa, Mohammed Al Sebayel, Khalid Al Kahtani, Faisal Aba Alkhail
Musthafa Chalikandy Peedikayil, Hamad Ibrahim Al Ashgar, Abdullah Al Mousa, Khalid Al Kahtani, Department of Medicine, King Faisal Specialist, Hospital and Research Center, Pin 11211, Riyadh, Saudi Arabia
Mohammed Al Sebayel, Faisal Aba Alkhail, Liver Transplantation and Hepatobiliary Surgery, King Faisal Specialist Hospital and Research Center, Pin 11211, Riyadh, Saudi Arabia
Author contributions: Peedikayil MC designed the study, collected data and wrote the paper; Al Ashgar HI, Al Mousa A, Al Sebayel M, Al Kahtani K and Alkhail FA contributed in writing the project and preparation of manuscript.
Correspondence to: Hamad Ibrahim Al Ashgar, MD, Department of Medicine, King Faisal Specialist, Hospital and Research Center, PO Box 3354, Pin 11211, Riyadh, Saudi Arabia. alashgar@kfshrc.edu.sa
Telephone: +966-1-4424729 Fax: +966-1-4427499
Received: August 1, 2012
Revised: November 8, 2012
Accepted: January 5, 2013
Published online: March 27, 2013
Processing time: 240 Days and 11.4 Hours
Abstract

AIM: To determine liver transplantation outcomes in Wilson’s disease (WD) patients, focusing on neurological manifestations.

METHODS: This retrospective study assessed data from 16 WD patients (nine males, 56%) who had liver transplants between 1991 and 2007. Survival, graft function, and neurological complications were assessed during a follow-up period of up to 15 years. In addition, each patient’s medical record was reviewed in detail to find the type of Wilson’s disease (hepatic or hepatic plus neurological WD), indication for liver transplantation, use of chelating agents prior to transplantation, immediate and long term complications following transplantation, the donor details, and the pathology of explanted liver.

RESULTS: End-stage liver disease was the indication for transplantation in all 16 WD patients. Four patients displayed WD-related neurological symptoms in addition to liver disease. Living-related liver transplantation was done in three cases. One patient died on postoperative day 6 due to primary graft non-function. One-year post liver transplant survival was 94%. Neurological manifestations of all four patients disappeared during their follow-up. Four patients developed acute cellular rejection, but all responded to treatment. One patient developed chronic ductopenic rejection after 15 years post-transplantation and their graft failed; this patient is currently waiting for re-transplantation. Fourteen patients (88%) are still living. The long-term average survival is currently 10.5 years, with a current median survival of 8 years. Long-term graft survival is currently 81%.

CONCLUSION: Short- and long-term survival in WD patient liver transplantation was excellent, and neurological and psychological WD manifestations disappeared during long-term follow-up.

Keywords: Wilson’s disease; Liver transplantation; Neurological; Psychiatric; Penicillamine; Saudi Arabia; Transplantation