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目的 :比较二巯丁二酸与青霉胺长期维持治疗肝豆状核变性 (HLD)的疗效。方法 :将 12 0例HLD分 2组。A组 6 0例用二巯丁二酸 750mg ,po ,bid ;B组 6 0例用青霉胺 2 50mg ,po ,qid ;维持治疗时间 6mo～ 3a ,平均 1.5a±s1.0a。分 6mo～ 2a和 >2a以改良Goldstein分级法判断疗效。结果 :A组 2个治疗时间段的总有效率分别为 80 %和 85% ,B组为 58%和 59% ,差异均有显著意义(P <0 .0 5) ;A ,B 2组无效病人互换药物治疗后有效率分别为 2 7%和 6 8% ;A ,B 2组不良反应发生率分别为 15%和 37% ,差异均有显著意义 (P <0 .0 5)。结论 :二巯丁二酸长期疗效优于青霉胺 ,尤其适用于青霉胺治疗无效者
Objective: To compare the efficacy of dimercaptosuccinic acid and penicillamine in the maintenance of hepatolenticular degeneration (HLD). Methods: 120 cases of HLD were divided into two groups. A group of 60 cases with mercaptosuccinic acid 750mg, po, bid; B group 60 cases with penicillamine 2 50mg, po, qid; maintenance treatment time 6mo ~ 3a, an average of 1.5a ± s1.0a. Points 6mo ~ 2a and> 2a to improve Goldstein classification to determine the efficacy. Results: The total effective rates of the two treatment periods in group A were 80% and 85%, respectively, and those in group B were 58% and 59%, respectively (all P <0.05); group A and B 2 were ineffective The effective rates of drug-swallowing patients were 27% and 68%, respectively. The incidence of adverse reactions in group A and B 2 were 15% and 37%, respectively, with significant differences (P <0.05). CONCLUSIONS: The long-term efficacy of DMT is better than that of penicillamine, especially for penicilline ineffective