Copper Deficiency in Liver Diseases: A Case Series and Pathophysiological Considerations

The role of copper in liver disorders is best recognized in Wilson’s disease, in which hepatic copper accumulation is not only pathognomonic, but also pathogenic as a source of cellular reactive oxygen species.(1) Elevated hepatic copper is also found in cholestatic liver diseases, but this is likely a consequence of decreased biliary excretion of copper and not a cause of the underlying liver disease.(2,3)

The opposite end of the spectrum (reduced hepatic copper concentration) is rarely reported in patients with liver diseases.(4) In mammals, cytochrome-c oxidase, ceruloplasmin, hephaestin, and copper-zinc superoxide dismutase represent a partial list of cuproenzymes whose metabolism and function depend on copper availability.(5-8)

In addition, copper plays a key role in the innate immune system, acting as a “bullet” for effective killing of bacteria and fungi by macrophages.(9) Consequences of copper deficiency include iron overload, tissue fibrosis, cytopenia, and susceptibility to infections.(10-14) Although these clinical features are also prevalent in advanced cirrhosis,(15-17) the significance of copper deficiency in liver disorders has only been reported in human fatty liver disease and preclinical animal models.(18-20)

 

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