活体肝移植治疗肝豆状核变性1例

A case of report on living donor liver transplantation for treatment of Wilson's disease

目的:探讨活体肝移植治疗小儿晚期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1例。结果:供体术后顺利康复;患儿术后健康存活,至今18个月,正常饮食,血铜蓝蛋白已恢复正常。结论:活体肝移植是治疗小儿晚期肝豆状核变性的有效方法,具有其它类型肝移植无可比拟的优越性。

Objective:To explore the efficacy of living donor liver transplantation for the treatment of children with Wilson's disease. Methods:A child aged 6 year-old with Wilson's disease was treated with living donor liver transplantation, clinical and biochemical monitoring, comprehensive therapy including infection control, immunosuppression, nutritional supporting. Results:The donor recovered uneventfully, the child has been surviving for 18 months. Her diet and serum ceruloplasmin level has been recovered to normal. Conclusion:Living donor liver transplantation has been proven to be effective therapy in children with Wilson's disease for its many unsurpassed advantages.