Penicillamine Therapy for Hepatolenticular Degeneration

Abstract

Ingestion of copper was reduced and its excretion was enhanced pharmacologically, principally by the administration of penicillamine, in 33 patients with hepatolenticular degeneration, of whom eight were asymptomatic. Concomitant clinical improvement was noted most markedly and regularly in the neurological manifestations of the disease, whereas the psychiatric and hepatic disturbances lessened in some individuals, but worsened in others. Clinical manifestations did not develop in the asymptomatic patients. The administration of penicillamine was occasionally accompanied by fever and several types of skin eruptions, and, less commonly, by hematological and renal reactions.