Gospe S M, Caruso R D, Clegg M S, Keen C L, Pimstone N R, Ducore J M, Gettner S S, Kreutzer R A
Departments of Neurology, Pediatrics, Radiology, Nutrition, and Internal Medicine, University of California, Davis, California, USA.
Arch Dis Child. 2000 Nov;83(5):439-42. doi: 10.1136/adc.83.5.439.
Progressive myelopathy is a rare complication of chronic hepatic disease which has never been reported in the paediatric age group. We describe the 11 year course of an adolescent male with hepatic myelopathy caused by cryptogenic micronodular cirrhosis. His condition has been associated with persistent polycythaemia and extraordinary increases of whole blood manganese, with magnetic resonance imaging evidence of manganese deposition within the basal ganglia and other regions of the brain. The patient has developed neither liver failure nor parkinsonism. The pathophysiological bases of this multiorgan system disorder are described.
进行性脊髓病是慢性肝病的一种罕见并发症，在儿童年龄组中从未有过报道。我们描述了一名患有隐源性微小结节性肝硬化所致肝脊髓病的青少年男性的11年病程。他的病情与持续性红细胞增多症以及全血锰异常升高有关，磁共振成像显示基底节和大脑其他区域有锰沉积。该患者既未出现肝衰竭也未出现帕金森综合征。本文描述了这种多器官系统疾病的病理生理基础。
