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目的观察谷胱甘肽片治疗肝豆状核变性的疗效及不良反应。方法 151例确诊患者常规服用葡萄糖酸锌和肝豆灵片,并随机分为3组:谷胱甘肽组(20mg·kg~(-1)·d~(-1))56例,二巯基丁二酸组(60mg·kg~(-1)·d~(-1))50例和青霉胺组(25mg·kg~(-1)·d~(-1))45例,疗程为12wk,治疗前后分别记录症状、体征和生化指标变化情况,观察不良反应。结果二巯基丁二酸组和青霉胺组24 h尿排铜量均较治疗前显著增高(P<0.01),青霉胺组白细胞数较治疗前降低(P<0.05);谷胱甘肽组异常的总胆红素和丙氨酸转氨酶在治疗后明显得以改善(P<0.05),24 h尿排铜量与治疗前相比无显著变化(P>0.05)。谷胱甘肽组治疗有效率为88%,不良反应发生率为4%;二巯基丁二酸组有效率82%,不良反应发生率18%;青霉胺组有效率89%,不良反应发生率38%。3组间疗效无显著差异(P>0.05),不良反应发生率有显著差异(P<0.05)。结论谷胱甘肽口服治疗肝豆状核变性疗效确切,安全性好。
Objective To observe the efficacy and adverse reactions of glutathione tablets in the treatment of hepatolenticular degeneration. Methods One hundred and fifteen patients with definite diagnosis were given zinc gluconate and gGG, and were randomly divided into three groups: 56 patients with glutathione (20 mg · kg -1 · d -1) Forty five patients (50 mg) and 25 mg · kg ~ (-1) d ~ (-1) of succinate group (60 mg · kg -1 · d -1) 12wk, before and after treatment were recorded symptoms, signs and biochemical changes, observe the adverse reactions. Results The amount of copper excreted by 24 hours and 24 hours after treatment in DMH group and penicillamine group was significantly higher than that before treatment (P <0.01), while the leukocyte count in penicillamine group was lower than that before treatment (P <0.05) The levels of total bilirubin and alanine aminotransferase were significantly improved after treatment (P <0.05), and there was no significant change in copper excretion at 24 h after treatment (P> 0.05). The effective rate of glutathione treatment was 88%, the incidence of adverse reactions was 4%; Dimercaptosuccinic group effective rate 82%, adverse reaction rate 18%; penicillamine effective rate 89%, adverse reactions occurred Rate of 38%. There was no significant difference in efficacy between the three groups (P> 0.05), and the incidence of adverse reactions was significantly different (P <0.05). Conclusion glutathione oral treatment of Wilson’s disease is effective and safe.