小儿肝移植的神经病理学。

W A Hall, A J Martinez
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引用次数: 22

摘要

我们回顾了35例儿童患者的临床病史和尸检记录(年龄从9个月到18岁),他们接受了原位肝移植,使用环孢素和皮质类固醇进行免疫抑制。死亡时,19例患儿(54%)患有脑病,16例患儿(46%)嗜睡或昏迷,10例患儿(29%)癫痫发作,10例患儿正常。35例患者尸检均发现神经病理病变。血管病变如梗死、缺血、血栓形成和出血是最常见的神经病理表现(86%),其次是感染过程(29%)。白色念珠菌(2例)和烟曲霉(3例)是仅有的致病菌,均可引起脑膜脑炎。阿尔茨海默II型星形胶质细胞是慢性肝病的一个特征性特征,是最常见的尸检发现(69%)。3例患儿脑桥中央髓鞘溶解,1例患儿基底动脉血栓形成。神经系统并发症及其随后的神经病理是儿童肝移植术后发病率和死亡率的重要原因。血管损伤、电解质异常和涉及中枢神经系统的感染与肝功能和维持移植物活力所必需的免疫抑制直接相关。只有术后持续观察,结合快速的内科和外科治疗,我们才有希望改善小儿肝移植术后的预后。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Neuropathology of pediatric liver transplantation.

We reviewed the clinical histories and autopsy records of 35 pediatric patients (ranging in age from 9 months to 18 years) who underwent orthotopic liver transplantation using ciclosporin and corticosteroids for immunosuppression. At the time of death, 19 children (54%) had encephalopathy, 16 (46%) were lethargic or in coma, 10 (29%) had seizures, and 10 were normal. Neuropathological lesions were found on postmortem examination in all 35 patients. Vascular lesions such as infarction, ischemia, thrombosis, and hemorrhage were the most common neuropathological findings (86%) followed by infectious processes (29%). Candida albicans (2 patients) and Aspergillus fumigatus (3 patients) were the only offending organisms identified, both causing meningoencephalitis. Alzheimer type II astrocytes, a characteristic feature of chronic liver disease, were the single most common autopsy finding (69%). Central pontine myelinolysis was seen in 3 children and basilar artery thrombosis affected 1 child. Neurological complications and their subsequent neuropathology are a significant cause of morbidity and mortality after pediatric liver transplantation. Vascular insults, electrolyte abnormalities, and infections that involve the central nervous system are directly related to liver function and the immunosuppression necessary to maintain graft viability. Only with continued observation after surgery combined with rapid medical and surgical treatment can we hope to improve the prognosis following liver transplantation in the pediatric population.

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