Copper

Copper is now well established as an essential trace element. The majority of copper in the circulation is bound to caeruloplasmin which is synthesised in the liver, and binds 6-8 atoms of copper per molecule. Caeruloplasmin is an acute phase protein and can increase greatly in response to infection, injury, chronic inflammatory conditions or steroid hormones (including pregnancy, the OC pill and HRT). Copper deficiency presents as a microcytic hypochromic anaemia with marked neutropenia. Frank dietary copper deficiency is uncommon but subclinical copper deficiency may be more widespread than was first thought. The rare sex linked recessive disorder Menke's 'steely hair' disease is characterised by a failure of intestinal copper absorption due to a defect in the gene coding for the intestinal copper transporter ATP7A. Wilson disease (hepatolenticular degeneration) is an autosomal recessive disorder due to defects in the gene coding for the copper transporter (ATP7B) in other cells. Copper a especially the brain. Children and adolescents presenting with a variety of hepatic symptoms often associated with a normocytic anaemia should be investigated for Wilson disease. The classical presentation in adults is of progressive neurological symptoms and characteristic copper deposits in the corneas (Kayser-Fleischer rings). Chronic poisoning with copper or inherited disorder (Wilson disease) accumulates in the liver and other tissues, leads to gross hepatic copper overload with severe liver disease in young children. Indian childhood cirrhosis has been ascribed to storage of drinking water/milk in copper vessels and there have been several cases of copper poisoning from well water.