Wilson's disease treatment by triethylene tetramine dihydrochloride (trientine, 2HCl): long-term observations.
Wilson's disease treatment by triethylene tetramine dihydrochloride (trientine, 2HCl): long-term observations.
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三乙烯四胺二盐酸盐（曲恩汀，2HCl）治疗威尔逊氏病：长期观察。
DOI：
10.1159/000457456
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发表时间：
1992
期刊：
影响因子：
--
通讯作者：
T. Hashimoto
中科院分区：
文献类型：
--
作者：
J. Morita;M. Yoshino;H. Watari;I. Yoshida;T. Motohiro;F. Yamashita;Y. Okano;T. Hashimoto
威尔逊氏病是一种常染色体隐性遗传疾病，其特征是有毒量的铜在体内积累。三乙烯四胺二盐酸盐（Trientine，2 HCl）是一种新的螯合剂，可有效去除过量的铜，但长期疗效尚未研究。在此，我们报告了2例不能耐受D-青霉胺的Wilson病患者使用曲恩汀超过8年的情况。除血清铁浓度下降外，未发现明显副作用，且无贫血临床症状。在稳定状态下的年度检查中，血清铜水平保持在20微克/100毫升以下。24小时尿铜排泄量低于使用D-青霉胺的结果，而停用曲恩汀5天后，基础铜排泄量维持在100微克/天以下。除延髓症状外，肝脏和神经系统表现均恢复，无任何初始恶化。
Wilson's disease is an autosomal recessive disorder characterized by an accumulation of a toxic amount of copper in the body. Triethylene tetramine dihydrochloride (trientine, 2HCl) is a new chelating agent that may be effective in the removal of excess copper but long-term efficacy has not yet been investigated. Here we report the use of trientine over more than 8 years in 2 patients with Wilson's disease who could not tolerate D-penicillamine. We found no significant side effect, except a decreased serum iron concentration without clinical symptoms of anemia. In annual examinations at a steady state, the serum copper levels remained below 20 micrograms/100 ml. The 24-hour urinary copper excretion was less than that found using D-penicillamine, while the basal copper excretion, after 5 days abstinence from trientine, was maintained below 100 micrograms/day. Both hepatic and neurological manifestations except bulbar symptoms were recovered without any initial deterioration.