COPPER-ASSOCIATED LIVER-DISEASE IN CHILDHOOD

Background/Aims: Indian childhood cirrhosis is associated with high li ver copper concentrations and progressive liver disease with a high mo rtality, Early treatment with penicillamine was found to reduce mortal ity and reverse liver damage, We aimed to define the clinical features of copper-associated liver disease outwith the Indian subcontinent an d encourage the earlier consideration of the syndrome in cryptogenic l iver disease. Methods: Three European children presented between 10 an d 29 months of age with abdominal distension, pyrexia and hepatospleno megaly, Over 1-5 weeks their condition deteriorated rapidly due to liv er failure, Two died within 2 months of onset and one received a succe ssful liver transplant, In two cases consideration of the diagnosis oc curred only on examination of the liver after orthotopic liver transpl ant or death, Light microscopy was used, with haematoxylin and eosin, reticulin and orcein stains, Tissue, plasma and water copper levels we re measured by flame atomic absorption spectrometry. Results: All had micronodular cirrhosis and severe hepatocellular necrosis with Mallory bodies and copious-orcein positive material, Liver copper concentrati ons ranged from 1100-1310 mu g/g dry weight, For two patients domestic water with high copper content had been used for the preparation of f eeds, No environmental source of excess copper could be identified in the third case. Conclusions: We suggest that the above condition, whic h is called Indian childhood cirrhosis in the Indian subcontinent and Copper Storage Disease elsewhere, would be better named 'Copper-Associ ated Liver Disease in Childhood', emphasising the need to consider thi s disorder in unexplained liver disease and to seek possible sources o f excessive copper intake. (C) Journal of Hepatology.