1病因与发病机制肝豆状核变性(HLD)于1912年由W ilson作了确切和详细的报道,故也称W ilson病(WD),它是一种遗传性铜代谢障碍所致的肝硬化和以基底节为主的脑部变性的常染色体隐性遗传性疾病。正常健康人的铜排泄量基本上与从食品中的吸收量相等,从而能维持机体铜代谢的平衡。而 1 etiology and pathogenesis Hepatolenticular degeneration (HLD) in 1912 by W ilson made detailed and detailed report, it is also known as W ilson disease (WD), which is a genetic copper metabolism disorders caused by the liver Sclerosis and basal ganglia brain degeneration autosomal recessive disease. Normal healthy people’s copper excretion is basically the same amount of absorption from the food, which can maintain the body’s metabolism of copper. and