Atypical copper cirrhosis in Indian children

Abstract

In addition to ten children with Wilson's disease and one with Indian childhood cirrhosis, nine Indian children, aged from 4 to 15 years, with cryptogenic cirrhosis had significant deposits of stainable copper in their hepatocytes. These nine children had normal or elevated serum caeruloplasmin levels, absence of Kayser-Fleischer rings and a history of sibling death owing to liver disease in four cases. Histologically, fatty change was absent from all the biopsies but Mallory's hyaline, pericellular fibrosis and ballooning of hepatocytes were present in some. Since these children did not conform to the accepted clinical or histological definitions of either Indian childhood cirrhosis or Wilson's disease, they were designated as having atypical copper cirrhosis. The relationship of this group of cases to other types of copper cirrhosis is unknown.