Tamura Sumihito, Sugawara Yasuhiko, Kishi Yoji, Akamatsu Nobuhisa, Kaneko Junichi, Makuuchi Masatoshi
Artificial Organ and Transplantation Division, Department of Surgery, Graduate School of Medicine, University of Tokyo, 7-3-1 Hongo, Bunkyo-ku, Tokyo, Japan.
Clin Transplant. 2005 Aug;19(4):483-6. doi: 10.1111/j.1399-0012.2005.00371.x.
Liver transplantation with liver grafts from deceased donors is the treatment of choice for patients suffering from Wilson's disease (WD) with end-stage liver disease. There are few reports, however, on the use of liver grafts from living-related donors for WD. Five (two pediatric and three adult recipients) underwent living-related liver transplantation (LRLT) for WD at the University of Tokyo. Two patients presented with fulminant hepatic failure with hemolysis, and the other three presented with decompensating cirrhosis, one with an overlapping neurologic WD. All recipients had a low serum ceruloplasmin level (median: 18 mg/dL), high urinary copper level (mean: 1119 microg/d), and presented with Kayser-Fleischer rings before transplantation. Although one patient died from early graft thrombosis unrelated to WD, the other four patients have shown an excellent long-term prognosis. Following successful transplantation, there was a significant reduction in urinary copper excretion (median: 64 microg/d) in all patients. The neurologic symptoms of WD in one patient, however, worsened after 2 months and gradually subsided, but not completely, over the 2-yr follow-up. For advanced liver failure in WD, we consider LRLT a valuable life-saving option. The improvement of neurologic symptoms, however, requires further evaluation.
采用已故供体的肝脏进行肝移植是患有威尔逊病（WD）并处于终末期肝病患者的首选治疗方法。然而，关于使用亲属活体供肝治疗WD的报道却很少。东京大学有5例患者（2例儿童和3例成人受者）接受了亲属活体肝移植（LRLT）治疗WD。2例患者表现为暴发性肝衰竭伴溶血，另外3例表现为失代偿性肝硬化，其中1例合并神经型WD。所有受者移植前血清铜蓝蛋白水平均较低（中位数：18mg/dL），尿铜水平较高（平均值：1119μg/d），并出现凯-弗环。尽管1例患者死于与WD无关的早期移植肝血栓形成，但其他4例患者显示出良好的长期预后。移植成功后，所有患者的尿铜排泄量均显著降低（中位数：64μg/d）。然而，1例患者WD的神经症状在2个月后恶化，并在2年随访期间逐渐缓解，但未完全消失。对于WD晚期肝衰竭患者，我们认为LRLT是一种有价值的挽救生命的选择。然而，神经症状的改善仍需进一步评估。
