原位活体肝部分移植术治疗肝豆状核变性

Partial orthotopic living liver transplantation for Wilson's disease

目的 探讨辅助性原位活体肝部分移植治疗肝豆状核变性 (HL D)的可行性 .方法 受体女性 ,2 0岁 ,O型血 ,因肝豆状核变性而接受辅助性原位活体肝部分移植 .供体男性 ,2 1岁 ,A型血 .手术切除受体原病肝左外叶 2 6 0 g,取供体左外叶肝脏 2 95 g原位移植于受体 .术前行血浆交换 ,术后以FK5 0 6、激素、环磷酰胺联合免疫抑制 ,手术前后辅以排铜药物治疗 .结果 患者已生存半年余 ,基本恢复正常生活 ,移植肝脏体积明显增大 ,肝功能正常 ,临床症状及生化指标均缓解 .结论 辅助性原位活体肝部分移植是治疗肝豆状核变性的有效方法 .

AIM To investigate the feasibility of auxiliary partial orthotopic liver transplantation (APOLT) from a living donor to treat Wilson's disease. METHODS The patient was a 20 year old girl with Wilson's disease, whose blood type was O. The donor was a man aged 21 and his blood type was A. The graft (295 g), left lateral lobe of donor's liver, was implanted after resection of the left lateral segment (260 g) of the native liver. Considering there was a different blood type between donor and recipient, the blood plasma exchange was performed to the recipient before operation. Immunosup pressive therapy was given with FK506, adrenocortical hormones and cytoxan after operation. As an auxiliary measure, the tablets were taken to lustrate copper. RESULTS So far the recipient has survived 6 mo with a normal life. The graft has enlarged distinctly in size and the function of liver is normal. The extrapyramidal symptom and bio chemical indicators is remitted. CONCLUSION APOLT is an effective therapy for Wilson's disease.