Basal Copper Excretion in Wilson's Disease.

The clinical manifestations of Wilson's disease are believed to be due to the accumulation of toxic amounts of copper within the body. The primary aim of treatment is to remove these, usually by giving chelating agents that will combine with the copper and be excreted in the urine. The most effective of these therapeutic agents is penicillamine (Walshe, 1960), and clinical improvement follows in a high proportion of cases treated with this compound. When the more obvious clinical signs and symptoms have improved or disappeared it is difficult to judge the effectiveness of long-term treatment or to control dosage, and an objective method of assessing the degree of copper depletion is needed. This would be particularly valuable in those cases that are diagnosed from the biochemical findings and the family history, and may be treated with chelating agents before the typical signs have developed. Warren and Broughton (1962) described a case of Wilson's disease that had been successfully treated with D-penicillamine for four years, and they found that the best biochemical index of long-term response to treatment was the basal copper excretion measured on urines collected after penicillamine had been stopped for several days. This paper describes some further basal copper excretion results on this case, and on five others with Wilson's disease, before and after treatment with D-penicillamine.