论文部分内容阅读
肝豆状核变性又称Wilson病,是先天性铜代谢障碍所致的常染色体隐性遗传病。铜主要沉积于肝脏、脑、角膜、肾脏等脏器,因此临床表现复杂,早期易误诊。为探讨肝豆状核变性患儿头颅CT检查与临床表现的特点,以降低误诊率,提高患儿生存质量,现将我院于1992～1998年收治的25例肝豆状核变性患儿头颅CT检查结果和临床表现分析如下。
Wilson’s disease, also known as Wilson’s disease, is an autosomal recessive disease caused by congenital copper metabolism. Copper is mainly deposited in the liver, brain, cornea, kidney and other organs, so the clinical manifestations of complex early misdiagnosis. In order to investigate the characteristics of head CT examination and clinical manifestations in children with hepatolenticular degeneration, in order to reduce the misdiagnosis rate and improve the quality of life of children, now in our hospital from 1992 to 1998 admitted 25 cases of Wilson’s disease patients with head CT examination results and clinical manifestations are as follows.