活体部分肝移植治疗伴有神经系统病变的肝豆状核变性

目的 探讨活体部分肝移植治疗伴有神经系统病变的肝豆状核变性(Wilson病)的价值。方法 自2001年1月至2003年3月我院为15例Wilson病患者施行活体部分肝移植术,其中5例患者伴有不同程度神经系统病变症状,对其术后铜代谢指标及神经系统恢复情况进行总结。结果 5例移植手术均为亲属供肝。4例为左叶供肝(含肝中静脉),1例为右叶供肝(不含肝中静脉),供肝／受体体重比例(GRWR)为0．79—1．08。4例患者术后恢复顺利,1例术后发生肝动脉栓塞行再次肝移植术,所有病例均生活良好,无原发病复发表现。术后随访2～16个月,其中3例患者术后锥体外系症状及语言和运动障碍明显好转。结论活体部分肝移植是治疗伴有神经系统病变肝豆状核变性的有效手段,术后铜蓝蛋白和血清铜恢复正常,神经系统症状和体征均有不同程度改善。

Living donor liver transplantation treatment of Wilson's disease complicated with neuropathy

OBJECTIVE: To investigate living donor liver transplantation for Wilson disease with neurologic features. METHODS: From Jan 2001 to Mar 2003, fifteen cases of living donor liver transplantation were performed for Wilson Disease (WD), five of those were complicated with neurologic features. A retrospective analysis was given for cooper metabolism and neurologic features. RESULTS: All operation were living related liver transplantation and donors were mothers. Four left lobes with hepatic middle vein and one right lobe without hepatic middle vein were harvested from donors, and graft volume to recipient body weight ratio was 0.79 approximately 1.08. One patient occurred hepatic artery thrombosis and performed retransplantation later, the other recipients recovered satisfactorily. All patients showed Extrapyramidal sign and three patients companying with language handicap and dyskinesia alleviated postoperation follow-up between 2 and 16 months. All recipients are alive and remain well, and none have developed signs of recurrent WD. CONCLUSION: Living donor liver transplantation is effective treatment for WD complicated with nervous system symptom, ceruloplasmin is normal and Kayser-Fleischer ring and nervous system symptom are to various extents.