Liver copper content in patients with inflammatory bowel disease and associated liver disorders

In 45 patients with inflammatory bowel disease (9 with Crohn's disease and 36 with ulcerative colitis) and associated liver disorders, increased liver copper content (above 100 microgram/g dry weight) was found in 14 (31%). These patients represented about 50% of the patients with either biliary cirrhosis or pericholangitis. Four of the patients had levels regarded as compatible with hepatolenticular degeneration (greater than 250 microgram/g dry weight). In patients with chronic active hepatitis or non-specific changes in liver tissue, normal levels were found. The patients with Crohn's disease also had normal levels. Plasma ceruloplasmin was normal or increased in all. Determination of urinary copper output gave little diagnostic information. Alkaline phosphatases were markedly increased in most of the patients with increased liver copper concentration. In patients with ulcerative colitis and enhanced alkaline phosphatases, elevated liver copper content should be suspected and chelation therapy should be considered.