Copper balance studies during the treatment of patients with Wilson's disease

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Copper balance studies on 2 patients with Wilson's disease before and during DL-penicillamine therapy and one normal control without DL-penicillamine showed that in both patients the intestinal copper absorption on a given diet was higher than that of the normal subject. Urinary copper excretion also was elevated in both patients. From the unchanged mean fecal copper excretion it appeared that ingestion of penicillamine had no effect on the intestinal copper absorption; urinary copper excretion was markedly elevated in both patients during penicillamine therapy. In the absence of penicillamine both patients exhibited marked positive copper balances on a daily diet containing less than 2 mg. of total copper. For one patient with Wilson's disease it was estimated that the maximum allowable daily copper intake to avoid a positive copper balance in the absence of binding agents was 0.6 mg. The normal control subject was in balance with a daily intake of 1.2 mg. of copper. These results support those of other workers and emphasize the importance of both a low copper diet and a vigorous copper removal regimen to maintain a significant negative copper balance in Wilson's disease therapy. The usefulness of copper balance studies to evaluate the success of copper removal therapy was discussed and a simplified procedure for estimation of copper balance outlined. Urinary copper excretion studies in one patient extending more than 1 year demonstrated a gradual reduction with time in the effectiveness of the penicillamine-induced cupriuresis. As found by other workers increased dosages of penicillamine were required to maintain a definite negative copper balance.