Environmental causes of copper toxicity should not be forgotten in familial presentations.

This case describes a 15-month boy with a 6-month history suggestive of liver disease. His similarly affected non-identical twin died and autopsy investigations were consistent with intrahepatic cholestasis, progressive liver damage complicated by cirrhosis/portal hypertension and vitamin D deficient rickets. Cholestasis is common in liver disease.1 Familial cholestasis can be limited geographically/ethnically: Greenland Eskimo cholestasis, North American Indian cirrhosis, Turkish non-syndromic paucity of interlobular bile ducts, childhood cirrhosis in Arab Israelis, Tyrolean infantile cirrhosis and Indian childhood cirrhosis (ICC). Hepatic copper accumulation is a hallmark of ICC and on autopsy excessive copper was evident. Traditionally, copper exposure was most significant when raw/unpasteurised milk was boiled in copper pots—copper/brass-vessel usage has stopped since linkage to ICC accounting perhaps …