EFFECT OF ESTROGENS ON COPPER METABOLISM IN WILSON'S DISEASE*

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Abstract
Ethinyl estradiol was administered to 11 patients with Wilson''s disease. In 4 patients the serum Cu and ceruloplasmin concentrations and the urinary Cu excretion increased. In 6 patients there was an increase in the serum Cu concentration, 3 of whom showed cupruresis. The levels of serum Cu and ceruloplasmin and the urinary Cu excretion showed no change in 1 patient. One patient improved clinically, 4 were unchanged, and 6 deteriorated during the estrogen administration. The interrelationships of total serum Cu, non-ceruloplasmin serum Cu, ceruloplasmin, urinary Cu excretion, and clinical change were investigated. Neither ceruloplasmin elevation nor cupruresis necessarily led to improvement. Cupruresis and clinical deterioration could, in general, be correlated with an increase in the non-ceruloplasmin fraction of serum Cu.