JACK B. STRONG JR.; HUGH DEMPSEY, M.D.; S. RICHARDSON HILL JR., M.D.
Hepatolenticular degeneration (HLD, Wilson's disease)1 is associated with abnormal deposition of copper in the brain, liver, kidneys, and other body tissues,2 apparently due to an inherited defect in copper metabolism.3 Clinically the disorder is manifested by signs and symptoms of basal ganglia disease, postnecrotic hepatic cirrhosis,4 Kayser-Fleischer rings,5 hypoceruloplasminemia, hypocupremia, hypouricemia, cupruresis, and aminoaciduria.6 In light of our present state of knowledge concerning the pathogenesis of this disease, rational therapy should aim at producing a negative copper balance. This may be done both by decreasing copper intake and absorption, and by removing the excess copper deposition in tissue before
STRONG JB, DEMPSEY H, HILL SR. THE SUCCESSFUL MANAGEMENT OF HEPATOLENTICULAR DEGENERATION WITH PENICILLAMINE: STUDIES ON THREE GENERATIONS OF A FAMILY12. Ann Intern Med. 1961;54:198–204. doi: 10.7326/0003-4819-54-2-198
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Published: Ann Intern Med. 1961;54(2):198-204.
Encephalopathy, Gastroenterology/Hepatology, Liver Disease, Neurology.
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Print ISSN: 0003-4819 | Online ISSN: 1539-3704
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