活体肝移植治疗肝豆状核变性5例

目的 探讨活体肝移植治疗小儿肝豆状核变性的临床疗效。方法 采用亲体部分肝移植术及术后监测临床生命体征、血生化指标,抗感染、免疫抑制、营养支持等综合方法,治疗肝豆状核变性5例。结果 供体术后顺利康复;患儿术后健康存活,至今分别为3(3例)、2、1年。肝脏功能、血铜蓝蛋白已恢复正常,正常饮食,均无复发。结论 活体肝移植可纠正小儿肝豆状核变性的肝脏病变及原有的代谢缺陷,提高患儿生活质量,挽救其生命,是治疗小儿肝豆状核变性终末期肝病的一种有效方法。

Living donor liver transplantation for Wilson′s disease in 5 children

Objective To explore the efficacy of living donor liver transplantation in the treatment of children with Wilson's disease who had severe hepatic insufficiency. Methods Five children(4 females; 1 male; age range 6 to 11 years ) with Wilson's disease were treated with living donor liver transplantation from mother donors, clinical and biochemical monitoring, comprehensive therapy including infection control, immunosuppression, and nutritional supporting. Results The donors recovered uneventfully and the five children survived for 5 years(3 children) ,2 years, 1 years. They remained well with normal liver function and no disease recurrence, and serum ceruloplasmin levels were normal (all >0.2 g/L)with normal diets. Conclusions Living donor liver transplantation for hepatic complications of Wilson's disease can cure and correct the underlying metabolic defect. It is a lifesaving therapy in children with fulminant Wilsonian hepatitis and has many unsurpassed advantages.