LONG-TERM TREATMENT OF WILSONS-DISEASE WITH TRIETHYLENE TETRAMINE DIHYDROCHLORIDE (TRIENTINE)

Long-term treatment with triethylene tetramine dihydrochloride, (trien tine, TETA) was evaluated in 19 patients with Wilson's disease (WD). T wo were given the drug as first choice and 17 after treatment with pen icillamine. The change was made because of side-effects, lack of impro vement or worsening of neurological symptoms. All penicillamine-induce d side-effects reverted, Thirteen patients still receive trientine, an d the mean total observation time on this treatment is 8.5 years/patie nt. Seven of the 13 are free from symptoms related to WD, five have mi ld to moderate neurological symptoms, mainly dysarthria. One patient w ith neurological symptoms who received trientine from the start of tre atment deteriorated rapidly and is now severely dystonic. The symptoms initially worsened and later improved in one patient. All other patie nts improved during trientine treatment. Three patients died: two from a multifocal cancer including the liver and one non-complier from a r uptured spleen. Two patients underwent liver transplantation for progr essive liver failure: one non-complier and one with liver cirrhosis wh ose liver function deteriorated despite treatment; both are now free f rom symptoms. Unexpectedly, two patients developed a serious colitis, one with duodenitis as well, that improved after withdrawal of the dru g. No other unfavourable effects of trientine were recorded.