Martin Adrian P, Bartels Michael, Redlich Jens, Hauss Johann, Fangmann Josef
Klinik für Visceral-, Universitätsklinik Leipzig, Transplantation-, Thorax- und Gefässchirurgie, Leipzig, Germany.
Clin Transplant. 2008 Mar-Apr;22(2):216-21. doi: 10.1111/j.1399-0012.2007.00777.x.
Wilson's disease is an inherited disorder of copper metabolism, presenting with prominent hepatic and neurologic manifestations. There is an established place for liver transplantation in the presence of liver disease, while the indication for neurologic manifestations is debated. Between 1993 and 2005, 11 patients were liver transplanted for Wilson's disease at our institution. We retrospectively reviewed the medical records of the patients. The pathology of the explanted livers was analyzed. The patients were divided into three groups based on the evolution of the disease. Postoperative data gathered included patient and graft outcome, complications, neurologic status, and copper metabolism. Six males and five females were transplanted at a mean age of 29.7 yr (range 15-48 yr). Three patients had a fulminant presentation, two patients had decompensation of established disease, and six patients had chronic disease. Neurologic features were prominent in five patients. The pathologic analysis of the explanted graft showed cirrhosis in all patients. The five patients with fulminant and acute on chronic presentations also showed necrosis in the explant. The mean postoperative follow-up was 56.8 months (range 10-129 months). Two patients were re-transplanted. One patient died because of severe sepsis. Two patients with severe neurologic dysfunction showed significant remission of symptoms. Liver transplantation is a safe and effective treatment for both acute and chronic presentations of Wilson's disease. Acute presentation correlates with the presence of necrosis in the explanted liver. In our series, there was a relevant improvement of the neurologic features after transplantation.
威尔逊病是一种遗传性铜代谢紊乱疾病，表现为明显的肝脏和神经症状。对于存在肝脏疾病的患者，肝移植有其既定的地位，而神经症状的肝移植指征则存在争议。1993年至2005年期间，我院有11例威尔逊病患者接受了肝移植。我们回顾性分析了这些患者的病历。对切除肝脏的病理进行了分析。根据疾病进展将患者分为三组。收集的术后数据包括患者及移植物结局、并发症、神经状态和铜代谢情况。共11例患者接受移植，其中男性6例，女性5例，平均年龄29.7岁（15 - 48岁）。3例患者为暴发性表现，2例为已确诊疾病失代偿，6例为慢性疾病。5例患者有明显的神经特征。切除移植物的病理分析显示所有患者均有肝硬化。5例暴发性及慢性急性发作的患者，其切除的肝脏也有坏死表现。术后平均随访56.8个月（10 - 129个月）。2例患者再次移植。1例患者因严重脓毒症死亡。2例严重神经功能障碍患者症状明显缓解。肝移植对于威尔逊病的急性和慢性表现都是一种安全有效的治疗方法。急性表现与切除肝脏中坏死的存在相关。在我们的系列研究中，移植后神经特征有显著改善。
