Is non-Indian childhood cirrhosis caused by excess dietary copper?

Indian childhood cirrhosis is generally believed to be caused by toxic excesses of hepatic copper derived from milk boiled in copper vessels. Sporadic cases of a disorder indistinguishable from Indian childhood cirrhosis have appeared in other countries where the toxic hepatic copper has been thought to be derived from drinking water. In published reports of seven 2-year-old or younger infants with non-Indian childhood cirrhosis (five of whom died), the copper content of their drinking water--which the authors considered the essential, if not the sole, aetiological factor--ranged from 0.05 to 6.8 mg Cu/L. We identified three Massachusetts towns in which between 1969 and 1991 there were 64,124 child-years of exposure of children under the age of 6 years to drinking water that contained between 8.5 and 8.8 mg Cu/L. Data from the Massachusetts Department of Public Health showed that there were 135 deaths among these children, but no deaths from cirrhosis or any form of liver disease. These data, and evidence of a genetic aetiology in three of the seven infants reported previously, suggest that non-Indian childhood cirrhosis is an inherited disorder.