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卡托普利和二巯基磺酸钠对肝豆状核变性患者的抗铜疗效

Anticopper efficacy of captopril and sodium dimercaptosulphonate in patients with Wilson's disease.

作者信息

Wang Xiao-Ping, Yang Ren-Min, Ren Ming-Shan, Sun Bo-Min

机构信息

College of Life Sciences, University of Science & Technology of China, Hefei, PR China.

出版信息

Funct Neurol. 2003 Jul-Sep;18(3):149-53.

Abstract

The aim of this study was to explore and compare initial treatment effects of captopril (Tensiomin) and sodium dimercaptosulphonate (DMPS) on a relatively large series of Wilson's disease inpatients. Two important markers of anticopper efficacy: serum sulphydryl and 24 h urinary copper levels in the patients were evaluated before and after treatment. The patients were randomly subdivided into 4 groups to allow statistical analysis (ANOVA) of the values recorded. The protocol was an open-label study of all the patients treated for 8 weeks (i.e., all the patients except those in the no-drug group), and a further six-month follow-up (post hospitalization) of the 14 patients administered captopril. Several copper-related variables were studied to evaluate the effect of the drugs on copper, and several biochemical and clinical variables were studied to evaluate potential toxic effects. Captopril was found to have a significant anticopper effect and did not markedly raise serum sulphydryl levels within this limited patient sample; the anticopper efficacy of captopril was, however, found to be markedly lower than that of DMPS; DMPS was found to raise the patients' serum sulphydryl and urinary copper levels. Evaluation of data from individual patients revealed evidence of a toxic side effect in only 1 patient, treated with DMPS, who exhibited transiently raised serum alanine aminotransferases, while no serious adverse events, upstanding syncope, irritating cough and leukopenia induced by captopril were noted. The results obtained in this four-group sample suggest that captopril might be a mild anticopper agent for Wilson's disease, possibly relieving the hepatic portal hypertension, but that DMPS has a greater field of anticopper efficiency than captopril. The authors also discuss recent experience of the overall treatment in China.

摘要

本研究旨在探讨并比较卡托普利(开博通)和二巯基丙磺酸钠(DMPS)对相对大量肝豆状核变性住院患者的初始治疗效果。评估了患者治疗前后抗铜疗效的两个重要指标:血清巯基和24小时尿铜水平。将患者随机分为4组,以便对记录的值进行统计分析(方差分析)。该方案是一项对所有接受8周治疗患者(即除无药物治疗组外的所有患者)的开放标签研究,以及对14例接受卡托普利治疗患者的进一步6个月随访(出院后)。研究了几个与铜相关的变量以评估药物对铜的影响,并研究了几个生化和临床变量以评估潜在的毒性作用。发现在这个有限的患者样本中,卡托普利具有显著的抗铜作用,且未明显提高血清巯基水平;然而,发现卡托普利的抗铜疗效明显低于DMPS;发现DMPS可提高患者的血清巯基和尿铜水平。对个体患者数据的评估显示,仅1例接受DMPS治疗的患者出现毒性副作用,表现为血清丙氨酸转氨酶短暂升高,而未观察到卡托普利引起的严重不良事件、直立性晕厥、刺激性咳嗽和白细胞减少。在这个四组样本中获得的结果表明,卡托普利可能是一种治疗肝豆状核变性的轻度抗铜药物,可能缓解肝门静脉高压,但DMPS的抗铜效率范围比卡托普利更大。作者还讨论了中国整体治疗的近期经验。

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