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 Objective: To compare the efficacy of sodium dimercaptopropane sulfonate and dimercaptosuccinic acid or penicillamine in the treatment of hepatolenticular degeneration (HLD). Methods: 130 HLD patients were randomly divided into 3 groups. A group of 50 patients (28 males and 22 females; age 19 ± s 5a) with sodium dimepulpustphane 1g, dissolved in 5% glucose injection 50 0mL, ivgtt, qd, intermittent 1d; Forty-two patients (21 males and 19 females; age 18 ± 4a) were treated with hypothyronuccinic acid 750 mg, po, bid, and 40 cases in group C (24 males and 16 females; age 16 ± 6 a) Penicillamine 2 50mg, po, qid. All use 4wk. To improve the Goldstein method to determine the efficacy. Results: The total effective rate in group A was 78% similar to that in group B (P> 0.05), but both of them were superior to those in group C (58%, P <0. 05) Lower than C group (P <0.05). CONCLUSIONS: Sodium dimercaptopropanesulfonate and dimercaptosuccinate are more effective and safe than penicillamine