In advanced Indian childhood cirrhosis (ICC) urine copper concentration was higher (range 416-103,448 mg/g creatinine) than in other hepatic diseases (range 67-10,303 mg/g creatinine). In early ICC urine copper concentration was more modestly raised (1188-9470 mg/g creatinine), but rose to high values (2222-42,819 mg/g creatinine) after a single dose of penicillamine 20 mg/kg. A post-penicillamine urinary copper:creatinine ratio greater than 10,000 mg/g supports a diagnosis of ICC. The concentration of copper in the hair, while increased in advanced ICC, is of no diagnostic value in early cases.
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