STUDIES ON COPPER METABOLISM. XVI. RADIOACTIVE COPPER STUDIES IN NORMAL SUBJECTS AND IN PATIENTS WITH HEPATOLENTICULAR DEGENERATION 1

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Abstract
One mg of Cu containing 1 mc of radio-activity was given orally to 4 normal subjects and 4 patients with Wilson''s disease. The same amount of Cu64 was injected intravenously into 4 normal subjects, 3 patients with Wilson''s disease, and 2 patients with alcoholic cirrhosis of the liver. The proportion of the administered Cu64 excreted in the urine and feces was determined and the uptake of the isotope into the albumin and globulin (ceruloplasmin) fractions of plasma was studied. The curve of disappearance from the plasma of 50 [mu]g of Cu64 after the administration was determined in 3 normal subjects, 4 patients with Wilson''s disease, and 2 patients with alcoholic cirrhosis of the liver. Cu64 uptake into the liver was measured with a body-surface scintillation counter in 2 normal subjects, 4 patients with hepatolenticular degeneration, and 2 patients with alcoholic cirrhosis of the liver. Following oral administration of Cu64 to normal subjects, an average of 0.1% of the administered dose was recovered in the urine and 72.4% in the stools; in the patients with Wilson''s disease, an average of 2.5% was recovered in the urine and 52% in the stools. The administration of K2S together with Cu64 to 2 of the patients with hepatolenticular degeneration resulted in an increase in the amount of activity recovered in the stools. Following intravenous administration of Cu64 to normal subjects, an average of 0.2% of the activity was recovered in the urine and 12.4% in the stools; in the patients with Wilson''s disease, an average of 5.4% was recovered in the urine and 2.5% in the stools. In the patients with alcoholic cirrhosis of the liver, an average of 0.8% was recovered in the urine and 8.5% in the stools. The uptake of Cu64 into ceruloplasmin was impaired in the patients with hepatolenticular degeneration, but not in the patients with alcoholic cirrhosis of the liver. The uptake of Cu64 by the liver was depressed in 3 of the 4 patients with hepatolenticular degeneration and in 1 of 2 patients with alcoholic cirrhosis of the liver. The uptake of Cu64 by the erythrocytes of patients with hepatolenticular degeneration was not different from that of the red corpuscles of normal individuals. These findings are interpreted as indicating that excessive accumulation of Cu in the tissues of patients is due primarily to increased absorption of Cu from the gastro-intestinal tract. Although a smaller proportion of the isotope administered intravenously was recovered in the stools of the patients with Wilson''s disease than was recovered in the stools of normal subjects, evidence presented supports the view that the excretion of Cu in the bile is probably not impaired. The absorption of cupric acetate can be effectively inhibited by the administration of K2S.