Bellary S, Hassanein T, Van Thiel D H
Division of Transplantation Medicine, University of Pittsburgh School of Medicine, Pennsylvania, USA.
J Hepatol. 1995 Oct;23(4):373-81. doi: 10.1016/0168-8278(95)80194-4.
BACKGROUND/AIMS: As has been the case with other metabolic diseases of the liver in the last decade, orthotopic liver transplantation has been applied to the treatment of Wilson's disease with increasing frequency. The experience at the University of Pittsburg with orthotopic liver transplantation for Wilson's disease is reported.
Between February 1981 and December 1991, 51 orthotopic liver transplants were performed on 39 patients (16 pediatric, 23 adults) with Wilson's disease. Twenty-two patients were transplanted because of a presentation co-existent with fulminant hepatic failure. Seventeen presented with chronic advanced liver disease with (n=9) or without (n=8) associated neurologic dysfunction.
The rate of primary graft survival (n-39) was 73% and patient survival was 79.4%. No patient mortality occurred beyond 3 weeks post-orthotopic liver transplantation. Survival was butter for those with a chronic advanced liver disease presentation (90%) than it was for those with a fulminant hepatic failure (73%) presentation, but the difference was not statistically significant.
背景/目的：在过去十年里，与其他肝脏代谢性疾病的情况一样，原位肝移植越来越频繁地应用于威尔逊病的治疗。本文报道了匹兹堡大学进行原位肝移植治疗威尔逊病的经验。
1981年2月至1991年12月，对39例威尔逊病患者（16例儿童，23例成人）进行了51例原位肝移植。22例患者因暴发性肝衰竭合并其他症状而接受移植。17例表现为慢性晚期肝病，其中9例伴有神经功能障碍，8例不伴有神经功能障碍。
原发性移植物存活率（n = 39）为73%，患者存活率为79.4%。原位肝移植术后3周后无患者死亡。慢性晚期肝病患者的存活率（90%）高于暴发性肝衰竭患者（73%），但差异无统计学意义。
1）目前，原位肝移植是暴发性肝衰竭型威尔逊病的首选治疗方法；2）对于无法进行其他治疗的晚期慢性肝病型威尔逊病患者，应考虑原位肝移植；3）原位肝移植仅部分纠正威尔逊病患者的潜在代谢缺陷，并将铜动力学从纯合子疾病患者的特征转变为杂合子携带者的特征，从而实现表型治愈。
