Disease Information for Vitamin E defic. in newborns

In general, tocopherols act as antioxidants to prevent lipid peroxidation of polyunsaturated fatty acids in cellular membranes;. The antioxidant activity of -tocopherol is similar to that of glutathione peroxidase, which contains selenium; the main manifestations of vitamin E deficiency are (1) mild hemolytic anemia associated with increased erythrocyte hemolysis and (2) spinocerebellar disease , which occurs mainly in children who have fat malabsorption due to abetalipoproteinemia, chronic cholestatic hepatobiliary disease, celiac disease, or a genetic abnormality in vitamin E metabolism; Retinopathy of prematurity, also called retrolental fibroplasia, may improve with vitamin E therapy, as may some cases of intraventricular and subependymal; and hemorrhage in the newborn; Infants are born in a state of relative vitamin E deficiency, with plasma -tocopherol levels below 5 µg/mL; The smaller and more premature the infant, the greater the degree of deficiency; Vitamin E deficiency in premature infants persists during the first few weeks of life and can be attributed to limited placental transfer of vitamin E, low tissue levels at birth, relative dietary deficiency in infancy, intestinal malabsorption, and rapid growth; As the digestive system matures, vitamin E absorption improves, and blood vitamin E levels rise; malabsorption generally underlies vitamin E deficiency; Genetic abnormality in the transport of vitamin E can also play a role; Hemolytic anemia in premature infants may be a manifestation of vitamin E deficiency; Such an infant has hemoglobin levels ranging from 7 to 9 g/dL, low plasma vitamin E levels, reticulocytosis, and hyperbilirubinemia; Children with chronic cholestatic hepatobiliary disease or cystic fibrosis manifest the neurologic syndrome of vitamin E deficiency; Its signs are spinocerebellar ataxia with loss of deep tendon reflexes, truncal and limb ataxia, loss of vibration and position sense, ophthalmoplegia, muscle weakness, ptosis, and Dysarthria; In adults with malabsorption, spinocerebellar ataxia due to vitamin E deficiency is extremely rare, no doubt because adults have large vitamin E stores in adipose tissue; In a rare genetic form of vitamin E deficiency without fat malabsorption, the liver appears to lack a protein that normally transfers d- -tocopherol from hepatocytes to very low density lipoprotein; Thus normal plasma -tocopherol levels cannot be maintained; Premature infants deficient in vitamin E have muscular weakness, creatinuria, and ceroid pigmentation with necrosis in muscle biopsies; Increased peroxide hemolysis is also observed; Plasma tocopherol levels are low; Excessive creatinuria and increased plasma creatine phosphokinase levels may be present in persons with vitamin E deficiency on a creatine-free diet; Large-caliber myelinated axons in the periphery may be lost and posterior columns in the spinal cord may degenerate in persons with spinocerebellar disease; [Merck Manual 17th]

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