首页> 外文期刊>Quarterly Journal of Medicine >Long-term treatment of Wilson's disease with triethylene tetramine dihydrochloride (trientine)
【24h】

Long-term treatment of Wilson's disease with triethylene tetramine dihydrochloride (trientine)

机译:三亚乙基四胺二盐酸盐(曲恩汀)长期治疗威尔逊氏病

获取原文
获取原文并翻译 | 示例
       

摘要

Long-term treatment with triethylene tetramine dihydrochloride, (trientine, TETA) was evaluated in 19 patients with Wilson's disease (WD). Two were given the drug as first choice and 17 after treatment with penicillamine. The change was made because of side-effects, lack of improvement or worsening of neurological symptoms. All peni-cillamine-induced side-effects reverted. Thirteen patients still receive trientine, and the mean total observation time on this treatment is 8.5 years/ patient. Seven of the 13 are free from symptoms related to WD, five have mild to moderate neurological symptoms, mainly dysarthria. One patient with neurological symptoms who received trientine from the start of treatment deteriorated rapidly and is now severely dystonic. The symptoms initially worsened and later improved in one patient. All other patients improved during trientine treatment. Three patients died: two from a multifocal cancer including the liver and one non-complier from a ruptured spleen. Two patients underwent liver transplantation for progressive liver failure: one non-complier and one with liver cirrhosis whose liver function deteriorated despite treatment; both are now free from symptoms. Unexpectedly, two patients developed a serious colitis, one with duodenitis as well, that improved after withdrawal of the drug. No other unfavourable effects of trientine were recorded.
机译:在19例威尔逊氏病(WD)患者中评估了三亚乙基四胺盐酸盐(trientine,TETA)的长期治疗。首选药物有两种,青霉素胺治疗后有17种。做出此更改是因为副作用,神经症状缺乏改善或恶化。所有由peni-cillamine引起的副作用都可以恢复。 13名患者仍接受曲恩汀治疗,这种治疗的平均总观察时间为8.5年/患者。 13种中有7种没有与WD相关的症状,其中5种有轻度至中度的神经系统症状,主要是构音障碍。从治疗开始就接受曲安汀治疗的一名神经症状患者迅速恶化,现在严重肌张力障碍。症状最初恶化,后来在一名患者中得到改善。 Trientine治疗期间所有其他患者均得到改善。 3例患者死亡:2例来自包括肝脏在内的多灶性癌症,1例因脾破裂导致不合规。 2例因进行性肝功能衰竭而接受肝移植:1例不合规和1例肝硬化,尽管接受治疗但肝功能恶化; 2例因肝功能不全而接受肝移植。两者现在都没有症状。出乎意料的是,有两名患者出现了严重的结肠炎,其中一名还患有十二指肠炎,在停药后情况有所改善。没有记录到曲安汀的其他不利影响。

著录项

相似文献

  • 外文文献
  • 中文文献
  • 专利
获取原文

客服邮箱:kefu@zhangqiaokeyan.com

京公网安备:11010802029741号 ICP备案号:京ICP备15016152号-6 六维联合信息科技 (北京) 有限公司©版权所有
  • 客服微信

  • 服务号