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Query: UMLS:C0022716 (Menkes)
 1,057 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Copper is an essential trace element that is required for a number of enzymes which are necessary for normal metabolic function. Metabolic balance studies have demonstrated that daily copper losses are approximately 1.3 mg/day. In order to remain in copper balance, the average adult male must consume a diet which contains at least 2 mg copper/day. It has been assumed that most diets satisfy this requirement because of the ubiquitous presence of copper in most foodstuffs. Recent studies, however, have shown that dietary copper may often fall below the estimated daily needs. Nevertheless, clinically evident copper deficiency has been documented in only a few situations. Of these disorders. Menkes' syndrome has been most intensively studied. This x-linked abnormality is associated with copper deficiency due to impaired gastrointestinal copper absorption. However, the clinical disorder cannot be corrected readily with copper replacement, thus suggesting that Menkes' syndrome may reflect more than simple copper deficiency. Nutritional copper deficiency appears to be well documented in two situations: (1) the newborn, usually premature, undergoing rapid growth on a diet poor in copper, and (2) the patient maintained on total parenteral nutrition for long periods of time without copper supplementation. In both of these situations, anemia and neutropenia are the most striking hematologic abnormalities associated with copper deficiency. Sideroblastic changes as well as nuclear maturation defects observed in erythroid precursors. However, suggest that there is an effect of copper deficiency on the hematopoietic system which cannot be explained solely by defective plasma iron transport.
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PMID:Copper deficiency in humans. 641 May 10

Animal and human studies have shown that copper is involved in the function of several enzymes. Studies have also shown that copper is required for infant growth, host defense mechanisms, bone strength, red and white cell maturation, iron transport, cholesterol and glucose metabolism, myocardial contractility, and brain development. Copper deficiency can result in the expression of an inherited defect such as Menkes syndrome or in an acquired condition. Acquired deficiency is mainly a pathology of infants; however, it has been diagnosed also in children and adults. Most cases of copper deficiency have been described in malnourished children. The most constant clinical manifestations of acquired copper deficiency are anemia, neutropenia, and bone abnormalities. Other, less frequent manifestations are hypopigmentation of the hair, hypotonia, impaired growth, increased incidence of infections, alterations of phagocytic capacity of the neutrophils, abnormalities of cholesterol and glucose metabolism, and cardiovascular alterations. Measurements of serum copper and ceruloplasmin concentrations are currently used to evaluate copper status. These indexes are diminished in severe to moderate copper deficiency; however, they are less sensitive to marginal copper deficiency. Erythrocyte superoxide dismutase and platelet cytochrome c activities may be more promising indexes for evaluating marginal copper deficiency.
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PMID:Copper as an essential nutrient. 861 66