Copper kinetics in infantile hepatobiliary disease

Copper metabolism was investigated in 33 infants with hepatobiliary disease. In 25 paients with biliary atresia, 37-135 days old, hepatic copper ranged from 5 to 133 microgram Cu/g wet weight (mean:43.3 microgram). In over two-thirds of the liver samples copper content was elevated above normal. There was no correlation between hepatic copper concentration and patients' age or degree of liver fibrosis. The mean hepatic copper content was also elevated in six infants with other hepatobiliary diseases (mean:41.6 microgram). Serial assays of copper excretion in bile were made in 25 infants having Kasai procedures for biliary atresia. The average daily excretion of biliary copper in 12 patients with successful operations was 3.3 to 33.7 microgram (mean:15.1 microgram), whereas in 13 patients without postoperative bile drainage, the daily values were 0.3-6.4 microgram (mean:2.7 microgram) (p < 0.0001). In five patients with active bile excretion who had repeated liver biopsies there was a steady decrease in hepatic copper concentration. The results indicate that derangement of copper homeostasis occurs frequently in infants with hepatobiliary disease and that in patients with biliary atresia successful reestablishment of bile flow effects a return toward normal copper metabolism probably because of enhanced biliary excretion.