Epstein O, Sherlock S
Gastroenterology. 1980 Jun;78(6):1442-5.
Triethylene tetramine dihydrochloride (trien) is a copper chelating agent used as the alternative drug of choice in the treatment of Wilson's disease. Because of its apparent safety, we have used the drug in 4 patients with primary biliary cirrhosis in whom penicillamine had to be withdrawn because of serious side effects. Trien is an effective cupruretic drug in primary biliary cirrhosis, but its use is limited by the occurrence of side effects that occurred in all 4 patients. Three patients developed gastrointestinal side effects, and one of these patients developed a skin rash. The 4th patient developed acute rhabdomyolysis within 48 hr of receiving the first dose of the drug. One patient tolerated therapy for 20 wk, and, although her liver copper concentration did not show a marked fall, aspartate transaminase levels fell, and her IgM concentration fell to normal. Trien is an unsuitable copper chelating drug in primary biliary cirrhosis, although it remains the alternative drug of choice in Wilson's disease.
三乙烯四胺二盐酸盐（trien）是一种铜螯合剂，用作治疗威尔逊氏病的替代首选药物。由于其明显的安全性，我们已将该药物用于4例原发性胆汁性肝硬化患者，这些患者因严重副作用而不得不停用青霉胺。Trien在原发性胆汁性肝硬化中是一种有效的排铜药物，但其使用受到副作用的限制，所有4例患者均出现了副作用。3例患者出现胃肠道副作用，其中1例患者出现皮疹。第4例患者在接受首剂药物后48小时内发生急性横纹肌溶解。1例患者耐受治疗20周，尽管她的肝铜浓度没有明显下降，但天冬氨酸转氨酶水平下降，IgM浓度降至正常。Trien在原发性胆汁性肝硬化中是一种不合适的铜螯合药物，尽管它在威尔逊氏病中仍然是替代首选药物。
