Hepatic Copper Content, Urinary Copper Excretion, and Serum Ceruloplasmin in Liver Disease

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Abstract
Increased liver Cu concentration and raised serum ceruloplasmin were demonstrated in primary biliary cirrhosis and disorders of the biliary tract and occasionally in chronic active hepatitis and cirrhosis of the liver. Of 13 patients with primary biliary cirrhosis 8 had liver Cu content as high as seen in patients with hepatolenticular degeneration (> 250 .mu.g/g dry wt). Normal liver content was found in patients with acute hepatitis, steatosis of the liver, hepatic amyloidosis, hemochromatosis and Gilbert''s syndrome. The urinary Cu excretion was increased (> 75 .mu.g/24 h) in half the patients with primary biliary cirrhosis and occasionally in the other patient groups. Serum ceruloplasmin was raised in more than half of all patients and none had levels below the reference range. Raised hepatic Cu content did not always coincide with enhanced urinary Cu excretion but was significantly correlated with this parameter and also with ceruloplasmin, alkaline phosphatases and vitamin-K-dependent clotting factors, but not with ALAT [alanine aminotransferase]. Combination of laboratory data, as found in typical cases of hepatolenticular degeneration, was not observed in this study, including 66 patients.