Zinc therapy in Wilson's disease: observations in five patients

Abstract

We report our experience with zinc (Zn) therapy in five patients with Wilson's disease (WD). In addition to neurologic examination, evaluation of Kayser-Fleischer rings and liver function tests, copper (Cu) and Zn concentrations in liver tissue, plasma, and urine were periodically evaluated by spectrophotometry. Many of the patients had had side effects due to penicillamine (PCA). Oral Zn sulphate (220 mg tid) reduced the WD symptoms and resulted in normal urinary Cu excretion in all five patients. One patient who had a transient gastric complaint during Zn administration, and in whom a decrease in liver Cu content was not observed, did not show any improvement in liver histology. He resumed PCA therapy after 29 months of Zn therapy. We conclude that long-term Zn treatment in Wilson's disease can be a safe and effective alternative to Cu chelating agents. However, patients should be periodically monitored for their Cu/Zn status to assess patient compliance with therapy.