目的:观察肝豆状核变性儿童铜代谢的变化及营养治疗的效果。方法:24例肝豆状核变性患儿,平均年龄9.38岁。入院前后检测血清铜、铜蓝蛋白含量及尿铜排出量,同时对受检儿童的父母进行血清铜蓝蛋白检测。结果:在24例患肝豆状核变性患儿中,87.6%的家庭父母双方对子女有不显性遗传。24例住院儿童采用驱铜药物及营养治疗控制后,病情缓解。血铜、铜蓝蛋白、尿铜在治疗前为:58.14+34.03μg/dl,125.51+64.03 mg/dl,289.70+168.96μg/dl;治疗后为80.65+32.26μg/dl,169.01+78.7 mg/dl,210.66+143.02μg/dl;P<0.05有显著性差异。结论:药物结合营养干预治疗意义显著,效果满意。 Objective: To observe the change of copper metabolism in children with hepatolenticular degeneration and the effect of nutrition treatment. Methods: 24 children with hepatolenticular degeneration, with an average age of 9.38 years. Serum copper, ceruloplasmin and urinary copper excretion were measured before and after admission. Serum ceruloplasmin was also detected in the parents of the tested children. RESULTS: Of the 24 children with Wilson’s disease, 87.6% of the parents in the family had no dominant inheritance of their children. 24 cases of hospitalized children with flooding copper drugs and nutrition treatment control, the disease eased. Blood copper, ceruloplasmin and urinary copper before treatment were: 58.14 + 34.03μg / dl, 125.51 + 64.03 mg / dl, 289.70 + 168.96μg / dl; after treatment they were 80.65 + 32.26μg / dl and 169.01 + 78.7 mg / dl, 210.66 + 143.02μg / dl; P <0.05 significant difference. Conclusion: The therapeutic effect of drug combined with nutrition intervention is significant and the effect is satisfactory.