二巯丙磺钠与二巯丁二酸或青霉胺治疗肝豆状核变性的比较

目的 :比较二巯丙磺钠与二巯丁二酸或青霉胺治疗肝豆状核变性 (HLD)的疗效。方法 :130例HLD病人随机分 3组。A组 50例 (男性 2 8例 ,女性 2 2例 ;年龄 19±s 5a)用二巯丙磺钠 1g ,溶于5%葡萄糖注射液 50 0mL ,ivgtt,qd ,每周间歇 1d ;B组 4 0例 (男性 2 1例 ,女性 19例 ;年龄 18± 4a)用二巯丁二酸 750mg ,po ,bid ;C组 4 0例 (男性 2 4例 ,女性 16例 ;年龄 16± 6a)用青霉胺 2 50mg ,po ,qid。均用 4wk。以改良Goldstein法判断疗效。结果 :A组总有效率 78%与B组的 75%相似 (P >0 .0 5) ,但均优于C组的 58% (P <0 .0 5) ,A ,B 2组不良反应低于C组 (P <0 .0 5)。结论 :二巯丙磺钠和二巯丁二酸较青霉胺更有效而安全

Comparison of therapeutic effects between unithiol, succimer and penicillamine on hepatolenticular degeneration

AIM: To compare the efficacies of unithiol (Uni) in treating hepatolenticular degeneration (HLD) with those of succimer (Suc) and penicillamine (Pen). METHODS: One hundred and thirty patients with HLD were randomly divided into 3 groups: (A) 50 patients (M 28, F 22; age 19±s 5 a) received Uni 1 g+5% glucose 500 mL, iv, drip, qd for 4 wk, there were 1 d interval between each week. (B) 40 patients (M 21, F 19; age 18±4 a) received Suc 750 mg, po, bid for 4 wk. (C) 40 patients (M 24, F 16; age 16±6 a) received Pen 250 mg, po, qid for 4 wk. Symptoms and therapeutic effects were assessed with Goldstein scale. RESULTS: The total response rates in group A 78%, was similar to that of group B 75% (P>0.05), but was better than that of group C 58%(P<0.05). The adverse reactions of group A or B were much less than those of group C (P<0.05). CONCLUSION: Uni and Suc are more effective and more safe than Pen in the therapy of HLD.