结论血铜含量及铜氧化酶含量对肝豆状核变性确诊有良好的指导意义，尿铜含量可帮助医生对临床疗效转归和用药选择做出正确评价。
Conclusion The content of serum copper and copper-oxygenase is of meaningful. The content assay of urine copper can help doctors choose drug and come to right evaluation.
方法：对临床及实验室检查证实的110例肝豆状核变性患者均进行颅脑ct平扫，部分给予增强扫描。
Methods: 110 patients with HLD confirmed by clinical and laboratory data were performed plain CT scanning, and some of them underwent additional contrast enhanced CT.
比较青霉胺及锌剂治疗肝豆状核变性（HLD）的疗效及对尿铜排泄的作用。
PURPOSE To compare the efficacy and urinary copper excretion in the treatment of hepatolenticular degeneration (HLD) with penicillamine and zinc.
目的评价MRI对诊断肝豆状核变性(HLD)的意义。
Objective to assess the significance of MRI in the diagnosis of hepatolenticular degeneration (HLD).
材料和方法：对临床和生化证实30例肝豆状核变性的儿童进行治疗前后的脑部及腹部的MRI检查。
Materials and Methods:Pretreatment and follow up brain and abdomen MRI were performed in30children with clinically-and biochemically-proved HLD.
材料和方法：对临床和生化证实30例肝豆状核变性的儿童进行治疗前后的脑部及腹部的MRI检查。
Materials and Methods:Pretreatment and follow up brain and abdomen MRI were performed in30children with clinically-and biochemically-proved HLD.
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