Peedikayil Musthafa Chalikandy, Al Ashgar Hamad Ibrahim, Al Mousa Abdullah, Al Sebayel Mohammed, Al Kahtani Khalid, Alkhail Faisal Aba
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.
World J Hepatol. 2013 Mar 27;5(3):127-32. doi: 10.4254/wjh.v5.i3.127.
To determine liver transplantation outcomes in Wilson's disease (WD) patients, focusing on neurological manifestations.
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.
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%.
Short- and long-term survival in WD patient liver transplantation was excellent, and neurological and psychological WD manifestations disappeared during long-term follow-up.
确定肝豆状核变性（WD）患者的肝移植结局，重点关注神经学表现。
这项回顾性研究评估了1991年至2007年间接受肝移植的16例WD患者（9例男性，占56%）的数据。在长达15年的随访期内评估生存率、移植肝功能和神经学并发症。此外，详细查阅每位患者的病历，以确定肝豆状核变性的类型（肝型或肝加神经型WD）、肝移植指征、移植前螯合剂的使用情况、移植后的近期和长期并发症、供体详情以及移植肝的病理情况。
所有16例WD患者的肝移植指征均为终末期肝病。4例患者除肝病外还表现出与WD相关的神经症状。3例进行了活体亲属肝移植。1例患者术后第6天因原发性移植肝功能衰竭死亡。肝移植后1年生存率为94%。所有4例患者的神经学表现在随访期间均消失。4例患者发生急性细胞排斥反应，但均对治疗有反应。1例患者在移植后15年发生慢性胆管减少性排斥反应，移植肝失功；该患者目前正在等待再次移植。14例患者（88%）仍存活。目前长期平均生存时间为10.5年，当前中位生存时间为8年。目前长期移植肝生存率为81%。
WD患者肝移植的短期和长期生存率良好，WD的神经学和心理学表现在长期随访期间消失。
