| Abstract: | Copper deficiency developed in a five-month-old prematurely born infant with an abdominal wall closure defect and a small bowel fistula under long-term parenteral nutrition. The first manifestations were typical bone changes including diffuse osteoporosis, delayed bone age, widened cupped metaphyses with beaks and a fracture, subperiosteal hematomas and ossifications in the shafts of long bones, and a diaphyseal fracture. Other findings that confirmed the diagnosis included edema of the limbs with pseudoparalysis, neurologic abnormalities, anemia, leukoneutropenia, and very low serum levels of copper and ceruloplasmin. Following initiation of copper supplementation, clinical, hematological and biological disorders resolved within a few days and roentgenologic bone abnormalities within four months. Copper deficiency develops only in the presence of specific risk factors, that are often combined in a single patient: inadequate stores due to prematurity, excessive loss due to chronic diarrhea, exudative enteropathy, a proximal stoma, or a biliodigestive fistula; and inadequate intake due to malabsorption or long-term exclusive parenteral nutrition (EPN). Appropriate copper supplementation is needed in all these high risk situations. Early, continuous copper supplementation is required for young children under EPN. Serum copper assays should be included among the periodically monitored biochemical parameters. |
