肝豆状核变性(hepatolenticu lar degeneration,HLD)是一种少见的常染色体隐性铜代谢障碍性疾病,临床较为少见,现报告1例如下。患者男,23岁。双手震颤半月余,服药无效来我院就诊。查体:双眼角膜见Kayser-F le ioker(K-F)环,指鼻试验阳性,肝肋下未及,无压痛。实验室检查:血清铜 Hepatolenticular degeneration (hepatolenticu lar degeneration, HLD) is a rare autosomal recessive copper metabolic disorders, the clinical relatively rare, 1 is reported as follows. Male patient, 23 years old. Two-hand tremor more than half a month, medication invalid to our hospital. Examination: Cornea see Kayser-F le ioker (K-F) ring, refers to the nasal test positive, liver ribs, and no tenderness. Laboratory tests: serum copper