PRESENT INTERPRETATION OF THE ROLE OF COPPER IN INDIAN CHILDHOOD CIRRHOSIS

A common killer disease of the past, Indian childhood cirrhosis (ICC), which became preventable and treatable in the early 1990s, is now rar e. ICC must be clearly distinguished in Indian children from other chr onic Liver disorders including Wilson disease. Grossly increased hepat ic, urinary, and serum copper concentrations are characteristic of ICC . These increased concentrations are easily demonstrated histologicall y with orcein-rhodanine staining. Environmental ingestion of copper ap pears to be the most plausible explanation for ICC, as shown by feedin g histories, the prevention of ICC in siblings and in the Pune distric t by a change in feeding vessels, and the dramatic reduction in incide nce of ICC throughout India. The nature and role of a second factor in the causation of ICC remains unclear, although an inherited defect in copper metabolism is strongly suspected. ICC, however, does not appea r to be a straightforward early onset of Wilson disease because cerulo plasmin is consistently normal and clinical and histologic recovery is maintained in the long term despite withdrawal of D-penicillamine the rapy. Descriptions of an ICC-like illness in the West suggest that dif ferent mechanisms (environmental, genetic, or both) can lead to the sa me end-stage liver disease: copper-associated childhood cirrhosis. ICC probably represents a specific form of copper-associated childhood ci rrhosis that requires high environmental copper ingestion for its full expression.