肝豆状核变性（HLD）是一种常染色体隐性遗传铜代谢障碍性疾病。现报道1例伴转氨酶高达1000u以上且持续半年的HLD，经中西医结合治验。1病例介绍患儿男，9岁。因体检发现肝功能异常6个月，动作欠灵活3个月于1996年4月28日转入。患儿半年前因右中耳炎住院发现谷丙转氨酶（ALT） Hepatolenticular degeneration (HLD) is an autosomal recessive genetic disorder of copper metabolism. Is reported in 1 case with transaminases up to 1000u and more than six months of HLD, the Chinese and Western medicine treatment. 1 case description Children male, 9 years old. Liver function abnormalities due to physical examination found 6 months, the action is not flexible 3 months on April 28, 1996 into. Six months ago in children with otitis media hospitalized found alanine aminotransferase (ALT)