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为总结儿童肝豆状核变性（ＨＬＤ）诊断经验与疗效，对６８例病儿进行临床分析，并对３１例用大剂量硫酸锌加小剂量青霉胺治疗者进行４～１１年随访。结果显示５８例（８５％）角膜Ｋ－Ｆ环阳性，５７例（８４％）血清铜氧化酶光密度减低，５９例（８７％）血铜降低，６５例（９６％）尿铜增加。随访显示８２％有症状病儿于治疗后６～１２个月内明显好转，全部病儿尿铜于２年内降至正常。表明角膜Ｋ－Ｆ环、铜氧化酶光密度、血及尿铜测定可使绝大多数病儿确诊，硫酸锌与青霉胺联合治疗ＨＬＤ安全有效。
In order to summarize the experience and curative effect of diagnosis of hepatolenticular degeneration (HLD) in children, clinical analysis was performed in 68 children and 31 cases were followed up for 4 to 11 years with high-dose zinc sulfate and low-dose penicillamine. The results showed that corneal K-F ring was positive in 58 cases (85%), optical density of 57 cases (84%) was decreased, blood copper decreased in 59 cases (87%) and urine copper increased in 65 cases (96%). Follow-up showed that 82% of symptomatic children were significantly improved within 6-12 months after treatment, urinary copper in all patients dropped to normal within 2 years. K-F ring cornea, optical density of copper oxidase, blood and urine copper determination of the vast majority of sick children can be diagnosed, zinc sulfate and penicillamine combined treatment of HLD safe and effective.