Wilson's Disease (Hepatolenticular Degeneration)

Twenty-eight patients with Wilson's disease (hepatolenticular degeneration [HLD]) who have been treated with penicillamine and a low-copper diet were studied. Four were asymptomatic siblings of known HLD patients, 3 other patients died after penicillamine and dietary therapy was instituted, and the remaining 21 patients have shown varying degrees of improvement. Copper balance studies at intervals of 6 or 12 months helped in assessing the copper balance and the need for altering the dose of penicillamine or the dietary copper content. Radioactive copper studies showed a slow increase in uptake by livers of symptomatic patients under long-term treatment. Prolonged freatment of the symptomatic patients seems to increase the hepatic capacity for trapping copper.