【摘要】 目的探讨肝移植治疗肝豆状核变性的疗效。方法 回顾性分析2003年9月至2009年7月接受肝移植手术治疗的5例肝豆状核变性患者的临床资料。结果 采用附加腔静脉整形的改良背驮式肝移植3例,成人间活体部分肝移植2例。围手术期死亡1例,死于肺部真菌感染和多器官功能衰竭,另4例患者恢复良好且存活,其中生存时间≥1年4例,≥3年3例。3例伴有神经精神功能障碍的患者中,除1例死亡外,另2例症状得到不同程度的改善。存活的4例术后1个月肝功能较术前明显改善,血清铜蓝蛋白水平明显升高。结论 全肝移植或活体部分肝移植术能改善肝豆状核变性患者的铜代谢和神经精神症状,提高患者的生活质量和存活率。更多还原

【Abstract】 Objective To evaluate the effect of liver transplantation for Wilson’s disease(WD).Methods The clinical records of 5 cases of WD who underwent liver transplantation from September 2003 to July 2009 were analyzed retrospectively.Results Three cases underwent modified piggyback liver transplantation with venacavaplasty and the other two cases underwent living-related liver transplantation.One patient died of fungal infection in the lung and multiple organ failure during perioperative period.Four patients recovered well with excellent life quality until now.Among 4 survived patients,all the patients have survived for more than 1 year and 3 patients have survived for more than 3 years.Among 3 patients with neuropsychic dysfunction,2 patients showed ameliorative neuropsychic symptoms with varying degrees after operation and 1 patient died.All the levels of liver function test and serum copper-protein of 4 survived patients recovered obviously in 1 month post-operation.Conclusion Orthotopic liver transplantation or living-related liver transplantation not only effectively improves copper metabolism of patients with WD and relieves their severe neurological manifestation,but also improves their life quality and survival rate.更多还原