Haslam R H, Sass-Kortsak A, Stout W, Berg M
Dev Pharmacol Ther. 1980;1(5):318-24.
Wilson's disease is an autosomal recessive disorder characterized by progressive cirrhosis or neurological signs. Early detection and prompt treatment can reverse the relentless course of the disease. Treatment with D-penicillamine substantially improves the outlook for such patients unless side effects preclude its use. We report the use of triethylene tetramine dihydrochloride (Trien), a new non-sulfhydryl-chelating agent, in a girl who was unable to tolerate D-penicillamine in spite of steroid coverage. The drug has been well tolerated without side effects for approximately 2 1/2 years. Our patient's favorable clinical response would suggest that Trien is a safe alternative agent for the treatment of Wilson's disease when D-penicillamine is not tolerated.
威尔逊氏病是一种常染色体隐性疾病，其特征为进行性肝硬化或神经症状。早期检测和及时治疗可逆转该病的无情病程。除非出现副作用而不能使用，用D-青霉胺治疗可显著改善此类患者的预后。我们报告了一名尽管使用了类固醇药物仍无法耐受D-青霉胺的女孩使用二盐酸三乙烯四胺（Trien）（一种新的非巯基螯合剂）的情况。该药耐受性良好，约2年半未出现副作用。我们患者良好的临床反应表明，当无法耐受D-青霉胺时，Trien是治疗威尔逊氏病的一种安全替代药物。
