Dahlman T, Hartvig P, Löfholm M, Nordlinder H, Lööf L, Westermark K
Department of Internal Medicine, University Hospital, Uppsala, Sweden.
QJM. 1995 Sep;88(9):609-16.
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 penicillamine-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.
对19例威尔逊病（WD）患者进行了二盐酸三乙烯四胺（曲恩汀，TETA）的长期治疗评估。2例患者将该药作为首选药物，17例患者在接受青霉胺治疗后改用该药。改用的原因是出现副作用、病情无改善或神经症状恶化。所有由青霉胺引起的副作用均已恢复。13例患者仍在接受曲恩汀治疗，每位患者的平均总观察时间为8.5年。13例患者中有7例没有与WD相关的症状，5例有轻度至中度神经症状，主要是构音障碍。1例从治疗开始就接受曲恩汀治疗的有神经症状的患者病情迅速恶化，现在严重肌张力障碍。1例患者症状最初恶化，后来有所改善。所有其他患者在曲恩汀治疗期间病情均有改善。3例患者死亡：2例死于包括肝脏在内的多灶性癌症，1例不依从治疗者死于脾破裂。2例患者因进行性肝衰竭接受了肝移植：1例不依从治疗者和1例尽管接受治疗但肝功能仍恶化的肝硬化患者；两人现在均无症状。出乎意料的是，2例患者出现了严重的结肠炎，其中1例还伴有十二指肠炎症，停药后病情有所改善。未记录到曲恩汀的其他不良影响。
