肝豆状核变性是一种铜代谢障碍的遗传病。国内外文献报道的病例绝大多数限于同胞发病,少数连续两代发病,我们在遗传咨询门诊遇到一个家系有6例发病,现报告如下。江氏二代共7人(见家系图),其中Ⅱ_3三年前死于本病,Ⅱ_16年前死于本病,其余Ⅱ_2、Ⅱ_4、Ⅱ_5均患此病,现正在治疗,其父亦患此病。父母非近亲结婚。先证者,女10岁。1986年11月就诊。患儿2年多来 Wilson’s disease is a genetic disorder of copper metabolism. The vast majority of cases reported in the literature at home and abroad are limited to the onset of siblings, a small number of consecutive two generations of incidence, we encountered in a genetic counseling clinic, a family of 6 cases, the report is as follows. Jiang’s second generation a total of 7 people (see family map), of which Ⅱ_3 died of the disease three years ago, died of Ⅱ Ⅱ 16 years ago this disease, and the remaining Ⅱ_2, Ⅱ_4, Ⅱ_5 are suffering from the disease, is being treated, and his father also suffering from This disease. Parents marry non-relatives. Probate, female 10 years old. 1986 November treatment. Children more than 2 years