二巯基丙磺酸钠和青霉胺治疗肝豆状核变性的驱铜效果对比分析

目的：对比分析青霉胺和二巯基丙磺酸钠治疗肝豆状核变性患者的驱铜效果及安全性，为临床更好地选择排铜药物提供参考依据。方法：36例肝豆状核变性患者随机分为两组，观察组16例给予二巯基丙磺酸钠治疗，对照组20例给予青霉胺治疗，两组均治疗4周。观察两组治疗前、后24h尿排铜量、临床表现及药物不良反应。结果：治疗后两组尿铜水平较治疗前明显升高，观察组尿铜水平（1375．98±582．09）μg/L]明显高于对照组（1016．05±444．11）μg/L]（t=2．106，P〈0．05）。观察组治疗中出现皮疹3例，食欲减退2例，血小板减少、白细胞降低、发热各1例；对照组出现皮疹8例，食欲减退6例，白细胞降低4例，丙氨酸氨基转移酶升高1例。结论：肝豆状核变性患者在治疗开始时可采用二巯基丙磺酸钠以加速铜的排泄，减轻游离铜对组织器官的损害，青霉胺口服治疗作为后续治疗药物。

Comparison of effects of DMPS and penicillamine on decoppering therapy of hepatolenticular degeneration

Objective:To assess the effects of DMPS and penicillamine on treatment of hepatolenticular degeneration and provide evidence for clinical drug selection.Methods:Thirty-six patients with Wilson's disease were randomly divided into two groups(group A and group B),16 patients in group A were treated with DMPS,20 patients in group B(control group)were treated with penicillamine,both groups were treated for 4 weeks.Quantity of 24h urinary copper,clinical manifestations and drug side effects were determined before and after treatment.Results:After treatment,urinary copper levels increased significantly in patients of both groups,urine copper level in patients of group A (1 375.98 ± 582.09)μg /L] was significantly higher than that of group B(1 016.05 ± 444.11)μg /L](t=2.106,P<0.05).In group A,three cases had rash,two cases had anorexia,thrombocytopenia,leukopenia and fever occurred in one case respectively;in group B,eight cases had rash,six cases had anorexia,four cases had leukopenia,alanine aminotransferase elevated in one case.Conclusion:Treatment of hepatolenticular degeneration with DMPS initially may speed up the excretion of copper,reduce free copper and reduce lesions of tissues and organs;penicillamine ingested orally can serve as following therapy.