American Journal of Clinical Nutrition, Vol 9, 695-699, Copyright © 1961 by The American Society for Clinical Nutrition, Inc.

Wilson's Disease

¹ From The Rockefeller Institute, New York, New York

Whatever the cause of Wilson's disease there is one clear cut biochemical abnormality: an increased total body copper. It seems therapeutically plausible to remove the excess copper as quickly and completely as possible. This can be achieved by oral administration of penicillamine. With judicious but humane advice concerning the avoidance of foods containing a high copper content and the regular administration of penicillamine, the copper content of the body returns toward normal and in some cases is associated with undoubted clinical improvement. Replacement of a low ceruloplasmin level either by estrogen administration or by infusion of ceruloplasmin seems of little value. The regular infusion of apoceruloplasmin, to prevent absorption of copper and penicillamine to eliminate the copper already present, would be logical therapy for this particular inborn error of metabolismn and already the therapeutic nihilism epitomized in the statement that inherited diseases are cured only by the grave is happily mistaken.

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