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二盐酸三乙烯四胺对威尔逊病铜代谢的作用机制

Mode of action of triethylenetetramine dihydrochloride on copper metabolism in Wilson's disease.

作者信息

Siegemund R, Lössner J, Günther K, Kühn H J, Bachmann H

机构信息

Paul Flechsig Institute for Brain Research, Department of Neuro-chemistry, Clinic of Leipzig University, Germany.

出版信息

Acta Neurol Scand. 1991 Jun;83(6):364-6. doi: 10.1111/j.1600-0404.1991.tb03964.x.

DOI:10.1111/j.1600-0404.1991.tb03964.x
PMID:1887758
Abstract

The drug of choice for the initial treatment of "decoppering" in Wilson's disease, an inherited disorder of copper metabolism, is the chelating agent D-penicillamine. In the case of harmful side-effects an alternative drug is triethylenetetramine dihydrocholoride (trien or trientine). Using the 24-h-urine excretion of copper and the oral copper loading test with copper-64, a double function for trien was found: trien increases the urine copper excretion and decreases the intestinal copper absorption respectively.

摘要

对于威尔逊病(一种遗传性铜代谢紊乱疾病)“去铜”初始治疗的首选药物是螯合剂D-青霉胺。若出现有害副作用,替代药物是二盐酸三乙烯四胺(trien或曲恩汀)。通过24小时尿铜排泄以及用铜-64进行的口服铜负荷试验,发现trien具有双重作用:trien分别增加尿铜排泄并减少肠道铜吸收。

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