Abnormalities of the Physiology of Copper in Wilson's Disease

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Abstract
Stool radioactive copper was decreased and urinary excretion increased after intravenous administration of cupric chloride67Cu in eight homozygotes of Wilson's disease; cumulative excretion for 10 to 14 days was 3.7% to 13.2% of the dose; in six heterozygotes cumulative excretion was 7.8% to 23.8% of dose. Cumulative excretion in normal subjects was 24% to 40% of the dose. Two control subjects with hepatic failure excreted 10% and 14% of the dose respectively. The excretory data agreed well with the whole-body retention measurements in all subjects. Intermittent sampling of duodenal contents during the first few days after intravenous administration of radioactive copper suggested reduced biliary excretion as the proximate cause of decreased stool radioactive copper in the wilsonian subjects.