Abstract
Raised hepatic copper in Indian Childhood Cirrhosis.
Percutaneous liver biopsies from 19 children with liver disease were studied. Indian Childhood Cirrhosis was diagnosed in 5, and in these orcein staining demonstrated widespread granular deposits within hepatocytes The hepatic copper content in these 5 cases was greatly increased at 1389 ± 525 μg/g dry weight (range 1045 - 2303 μg/g, normal range 15 - 55 μg/g) The other 14 children had various hepatic disorders. None had widespread granular orcein staining Hepatic copper concentrations were normal in 12, and slightly elevated in 2 (170 and 292 μg/g). The absence of prolonged jaundice, histological cholestasis, or serum lipoprotein X in the cases of ICC indicated that hepatic copper accumulation was not secondary to cholestasis Serum ceruloplasmin concentrations were greater than 20 mg/dl in all 19 cases. A survey failed to detect any cases of ICC in Asian children born in the UK, so implicating environmental factors. We suggest that copper is implicated in the aetiology of ICC, that early treatment with penicillamine may be effective,and that reduction of copper intake may be preventative.
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Tanner, M., Portmann, B., Mills, C. et al. Raised hepatic copper in Indian Childhood Cirrhosis: 68. Pediatr Res 14, 176 (1980). https://doi.org/10.1203/00006450-198002000-00095
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DOI: https://doi.org/10.1203/00006450-198002000-00095
