Pathomorphology of the liver in exogenic infantile copper intoxication in Germany

Pathomorphology of the liver has been reviewed in twelve German infants with chronic exogenic copper intoxication. Eight cases suffered from florid Indian childhood cirrhosis. Seven of these children died because of liver failure. One child received liver transplantation. In contrast, four children with a stable clinical course had a complete micronodular cirrhosis with only slight developed features of liver cell damage and stable clinical course. The copper content varied between 541 microg/g dry weight (norm <59 microg/g) and 698 microg/g fresh weight (norm <5 microg/g). There was no significant difference in the copper content between the children with florid Indian childhood cirrhosis and those with micronodular cirrhosis. The results show that there exists a spectrum of pathomorphological alterations in exogenic infantile copper disease correlating with the clinical outcome in contrast to the copper content of the liver. Copper intoxication of the liver should be of diagnostic concern in any unclear case of micronodular cirrhosis in early infancy.