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  • Title: Wilson's disease treatment by triethylene tetramine dihydrochloride (trientine, 2HCl): long-term observations.
    Author: Morita J, Yoshino M, Watari H, Yoshida I, Motohiro T, Yamashita F, Okano Y, Hashimoto T.
    Journal: Dev Pharmacol Ther; 1992; 19(1):6-9. PubMed ID: 1307347.
    Abstract:
    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.
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