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Treatment With Penicillamine and Changes in Hepatic Trapping of Radioactive Copper

Norman P. Goldstein, MD; W. Newlon Tauxe, MD; John T. McCall, MD; Raymond V. Randall, MD; John B. Gross, MD

Arch Neurol. 1971;24(5):391-400.

Abstract
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.

Author Affiliations
Rochester, Minn

From the departments of neurology (Dr. Goldstein), clinical pathology (Dr. Tauxe), and biochemistry (Dr. McCall), and the divisions of endocrinology and internal medicine (Dr. Randall) and gastroenterology and internal medicine (Dr. Gross), Mayo Clinic and Mayo Foundation, Rochester, Minn.

Footnotes
Accepted for publication Nov 27, 1970.

Read before the meeting of the American Neurological Association, Los Angeles, June 9, 1969.

Reprint requests to Mayo Clinic, 200 First St SW, Rochester, Minn 55901 (Dr. Goldstein).

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