Wang Xue-hao, Cheng Feng, Zhang Feng, Li Xiang C, Kong Lian B, Li Guo Q, Li Jun, Qian Xiao F
Liver Transplantation Center, First Affiliated Hospital of Nanjing Medical University, Nanjing, Jiangsu Province, China.
Transpl Int. 2005 Jun;18(6):651-6. doi: 10.1111/j.1432-2277.2004.00074.x.
Orthotopic liver transplantation has been applied to the treatment of Wilson's disease (WD), living-related liver transplantation (LRLT) has also been indicated for WD with increasing frequency. Between January 2001 and November 2003, 22 LRLTs were performed on patients (19 pediatric, three adults) with WD in liver transplantation center. Two patients were transplanted because of a presentation coexistent with fulminant hepatic failure. Twenty presented with chronic advanced liver disease with (n = 9) or without (n = 11) associated neurologic manifestations. All the recipients had low serum ceruloplasmin levels with a mean value of 12.8 +/- 3.2 mg/dl before transplantation and increased to an average of 26.0 +/- 3.6 mg/dl after LRLT at the latest evaluation. The survival patients with neurologic manifestations such as tremor, dysarthia, dysphagia, dystonia and sialorrhea had improved after LRLT. This suggests that LRLT not only resolves the hepatic but also ameliorates the neurologic consequences of WD.
原位肝移植已应用于威尔逊病（WD）的治疗，亲属活体肝移植（LRLT）也越来越频繁地被用于治疗WD。2001年1月至2003年11月期间，在肝脏移植中心对22例患有WD的患者（19例儿童，3例成人）进行了亲属活体肝移植。2例患者因暴发性肝衰竭并存的表现而接受移植。20例表现为慢性晚期肝病，伴有（n = 9）或不伴有（n = 11）相关的神经学表现。所有受者移植前血清铜蓝蛋白水平均较低，平均值为12.8±3.2mg/dl，在最近一次评估时，亲属活体肝移植后平均升至26.0±3.6mg/dl。有震颤、构音障碍、吞咽困难、肌张力障碍和流涎等神经学表现的存活患者在亲属活体肝移植后有所改善。这表明亲属活体肝移植不仅能解决肝脏问题，还能改善WD的神经学后果。
