摘要
目的探讨Wilson病患者接受肝移植治疗后的效果及预后。方法回顾性分析2002年10月-2008年8月因Wilson病在第二军医大学附属东方肝胆外科医院接受肝移植手术的8例患者的临床资料,其中男3例,女5例,年龄为12～42岁,平均年龄为(22.6±10.3)岁。术前所有患者的血清铜蓝蛋白和血铜水平均低于正常值(血清铜蓝蛋白为200～600mg/L,血铜为11～24.4μmol/L),其中5例术前存在神经病学症状。结果 5例患者接受原位肝移植术,3例接受活体肝移植术。除1例患者于术后1个月死于急性肝脂肪变肝功能衰竭外,其余患者术后肝功能均恢复顺利。术后随访35～124个月,平均随访时间为(79.7±20.3)个月。与术前比较,术后1个月7例存活患者总胆红素水平显著下降(P<0.05),白蛋白、血清铜蓝蛋白水平及血小板计数均显著升高(P值均<0.05);血铜水平有升高趋势,但差异无统计学意义(P>0.05);合并的神经病学症状得到不同程度的改善甚至消失;所有患者术后12个月内K-F环均消失。结论通过全肝移植或活体肝移植术,能改善Wilson病患者铜代谢,并可显著缓解神经病学症状。
Objeetive To determine the efficacy and prognosis of liver transplantation for Wilson＇ s disease （WD）. Methods From October 2002 to August 2008, 8 cases （3 men and 5 women） with a mean age of 22.6 years （12- 42 years） underwent liver transplantation for WD in our department. All the recipients had low serum levels of ceruloplasmin and copper before transplantation, and 5 of them demonstrated the neurological manifestations. Results There were 3 orthotopic liver transplantation （OLT） and 2 living donor liver transplantation （LDLT） in this study. Seven recipients recovered satisfactorily with excellent liver functions, while 1 patient died of acute liver steatosis in 1 month after transplantation. Discharged patients were well followed up for a mean duration of 79.7 months （35 - 124 months）. Compared to the levels before transplantation, the total bilirubin levels were significantly declined （P〈 0. 05）, the serum levels of albumin, ceruloplasmin and platelet were obviously increased （all P〈0.05）, while that of copper was increased though no significant difference was found （P〉0.05）. The neurological manifestations were also partly relieved or disappeared after liver transplantation. No K-P loop was found in the patients 12 months after operation. Conclusion OLT and LRLT are effective treatments for patients with WD,which not only improving their copper metabolism but also significantly relieving the severe neurological manifestations and improving life quality. （Shanghai Med J, 2011, 34; 656-659）
出处
《上海医学》
CAS
CSCD
北大核心
2011年第9期656-659,共4页
Shanghai Medical Journal
基金
上海市科学技术委员会医学引导项目(10411963200)
关键词
原位肝移植
活体肝移植
WILSON病
Orthetopic liver transplantations Living donor liver transplantations Wilson＇s disease
