肝豆片Ⅰ号治疗肝豆状核变性临床疗效观察

目的：观察肝豆片Ⅰ号治疗肝豆状核变性（ＨＬＤ）临床疗效及对其２４ｈ尿微量元素的影响。方法：口服肝豆片Ⅰ号治疗３４例ＨＬＤ４周，密切观察患者症状、体征及日常生活的变化，并测定患者治疗前及治疗后每周２４ｈ尿微量元素含量。结果：总有效率为７０．５９％，显效率为８．８２％。对儿童及（或）病情较轻的患者疗效较好。治疗后每周的２４ｈ尿排铜量均较治疗前显著增加（Ｐ＜０．０１）；虽然其２４ｈ尿排铜量和疗程是负相关（ｒ＝－０．９６；Ｐ＜０．０５），但是治疗后各周之间的２４ｈ尿排铜量两两比较无明显差异（Ｐ＞０．０５），故其尿排铜作用无减弱。结论：肝豆片Ⅰ号治疗ＨＬＤ有效，能促进ＨＬＤ尿铜的排泄，且未见明显毒副作用。

英文摘要:

Objective: To investigate the efficacy of HLD-Tablet I in treating hepatolenticular degeneration (HLD) and its effect on urinary trace element in 24 hours. Methods: After 4 weeks of treatment on 34 cases of HLD by oral taken HLD -Tablet I, the clinical practice was closely observed on the patients’ change of spoptoms, signs and daily life pattern, and measured the level of urinary trace element in 24 hours before the therapy and after that every week. Results: The total effective rate reached 70.59 %, marked effective rate was 8.82 %. The effect of treatment was better in the infantile and mild patient. After treatment, the daily output of urinary copper was obviously increased every week than that before (P <0.01 ). It was highly negative linear correlated with the duration of treatment (r = -0.96, P <0.05 ). But it was insignificantly different between any two weekly excretions of urinary copper per 24 hours after treatment by the HLD-Tablet I (P >0.05), so its effect on urinary copper excretion was not decreased. Conclusion: The HLD-Tablet I could increase the output of urinary copper, hence it was effective in treating HLD without apparent toxic or side reaction.

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