【摘要】 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。更多还原

【Abstract】 AIM To observe the efficacy and safety of glutathione tablets in the treatment of hepatolenticular degeneration.METHODS All confirmed 151 patients with hepatolenticular degeneration treated with zinc gluconate and Gandouling tablet were randomly divided into three groups:56 for GSH group(glutathione 20 mg·kg^-1·d^-1,po),50 for DMSA group(dimercaptosuccinic acid 60 mg·kg^-1·d^-1,po),and 45 for PCA group(penicillamine 25 mg·kg^-1·d^-1,po).All patients were treated for 12 weeks,clinical data were recorded and adverse reactions were observed before and after the treatment.RESULTS The 24 h urinary copper contents in DMSA group and PCA group after the treatment were significantly higher than those before the treatment（P<0.01）,and the white blood cell count in PCA group was lower than that before the treatment（P<0.05）.In the GSH group,the total bilirubin and alanine aminotransferase abnormality indicators were restored after the treatment（P<0.05）with no significant difference in 24 h urinary copper content（P>0.05）.The efficacy rate and adverse reaction rate were 88%and 4%in the GSH group,82%and 18%in the DMSA group,89%and 38%in the PCA group,respectively.There was no significant difference in efficiency among the three groups by the Ridit analysis（P>0.05）,but the adverse reaction rates among them had statistically significant differences （P<0.05）.CONCLUSION The effect of glutathione tablets in the treatment of hepatolentcular degeneration is reliable and secure.更多还原