Department of Gastroenterology, University Hospital Heidelberg, Heidelberg, Germany.
Clin Transplant. 2013 Nov-Dec;27(6):914-22. doi: 10.1111/ctr.12259. Epub 2013 Oct 9.
Wilson disease (WD) is an autosomal recessive copper storage disease resulting in hepatic and neurologic dysfunction. Liver transplantation is an effective treatment for fulminant cases for patients with chronic liver disease. Reports on the outcome of neuropsychiatric symptoms after orthotopic liver transplantation (OLT) are limited.
To assess the course of neuropsychiatric and hepatic symptoms after liver transplantation for Wilson disease
Nineteen patients with Wilson disease received liver transplantation and were followed prospectively from 2005 to 2010 for the development of hepatic, neurological and psychiatric symptoms.
Eight patients (all female) were transplanted for acute liver failure and eleven patients for chronic liver failure. Patient survival rates one and five yr after transplantation were 78% and 65%, respectively. Of the surviving patients, hepatic symptom scores improved in all patients and neurological symptom scores improved in all but one patient after OLT compared to the time of initial diagnosis and compared to pre-OLT status. Psychiatric symptoms showed moderate improvements.
Survival after OLT for Wilson disease with end-stage liver disease is excellent. Overall, neuropsychiatric symptoms improved after transplantation, substantiating arguments for widening of the indication for liver transplantation in symptomatic neurologic Wilson disease patients with stable liver function.
威尔逊病（WD）是一种常染色体隐性遗传性铜蓄积病，可导致肝和神经功能障碍。肝移植是治疗慢性肝病患者暴发性病例的有效方法。关于肝移植后神经精神症状结局的报告有限。
评估肝移植治疗威尔逊病后神经精神和肝脏症状的病程。
19 例威尔逊病患者接受肝移植，并于 2005 年至 2010 年进行前瞻性随访，以了解肝脏、神经和精神症状的发展情况。
8 例患者（均为女性）因急性肝功能衰竭，11 例患者因慢性肝功能衰竭而接受肝移植。移植后 1 年和 5 年的患者生存率分别为 78%和 65%。在存活患者中，与初始诊断时和移植前相比，所有患者的肝脏症状评分在 OLT 后均有所改善，除 1 例患者外，所有患者的神经症状评分均有所改善。精神症状有中等程度的改善。
终末期肝病患者行肝移植治疗威尔逊病的存活率非常高。总的来说，移植后神经精神症状有所改善，这为在稳定肝功能的有症状神经威尔逊病患者中扩大肝移植的适应证提供了依据。
