MENKES disease due to defective absorption of:
Correct Answer: Copper
Description: Ans: C (Copper) Ref: Harrison's Principles of Internal Medicine. 17th edition. 2008. Chapter 71. PgA49Explanation:Dietary copper deficiency is relatively rare, although it has been described in premature infants wdio are fed milk diets and in infants with malabsorption.Copper-deficiency anemia has been reported in patients with malabsorptive diseases and nephrotic syndrome and in patients treated for Wilson's disease with chronic high doses of oral zinc, which can interfere with copper absorption.Menkes' kinky hair syndrome is an X-linked metabolic disturbance of copper metabolism characterized by mental retardation, hypo- cupremia and decreased circulating ceruloplasmin.It is caused by mutations in the copper-transporting ATP7A gene.Children with this disease often die within 5 years because of dissecting aneurysms or cardiac rupture.Aceruloplasminemia is a rare autosomal recessive disease characterized by tissue iron overload, mental deterioration, microcytic anemia, and low serum iron and copper concentrations.The diagnosis of copper deficiency is usually made on the basis of low serum levels of copper (<65 microg/dL) and low ceruloplasmin levels (<20 mg/dL).Serum levels of copper may be elevated in pregnancy or stress conditions since ceruloplasmin is an acute-phase reactant and 90% of circulating copper is bound to ceruloplasmin.
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