Disease Information for Vitamin E defic. in newborns

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Clinical Manifestations
Signs & Symptoms
Muscle weakness
Ataxia
Ataxia, Cerebellar type
Awkward Uncoordinated Child
Hyporeflexia/DTRs decreased
Limb Ataxia
Limb ataxia/clumsiness child
Loss position sense/postural sense
Neurologic manifestations/signs
Position sense decreased/proprioception
Staggering Gait
Truncal ataxia
Unsteadiness movement/Gait in a child
Vibratory sense decreased
Vibratory sense decreased Legs
Weakness
Eyelid Retraction
Proptosis,bilateral
Clinical Presentation & Variations
Anemia in Children
Demographics & Risk Factors
Population Group
Child
Infant
Population/Pediatrics population
Population/Third world countries
Sex & Age Groups
Population/Child
Population/Child-Infant Only
Population/Children/all
Population/Fetal-newborn
Population/Infant
Population/Infant One Month old
Population/Neonate-newborn
Laboratory Tests
Microbiology & Serology Findings
Reticulocytosis
Abnormal Lab Findings - Decreased
Factor 2/Prothrombin (Lab)
Hematocrit (Lab)
Hemoglobin (Lab)
Niacin/Nicotinic Acid serum (Lab)
Abnormal Lab Findings - Increased
CPK/CK/Creatine kinase/Creatine phosphokinase (Lab)
Polychrome RBCs (Lab)
Reticulocytes (Lab)
URINE Creatine
Diagnostic Test Results
Pathology
BX/Muscle Biopsy Abnormal
BX/Muscle biopsy/Ceroid deposition
Associated Diseases & Rule outs
Rule Outs
Hemolytic disease of newborn
Associated Disease & Complications
Anemia in a Baby/Young child
Cerebellar ataxia
Congenital/Neonatal Anemia
Edema of newborn
Exophthalmos
Hemolytic disease of newborn
Ophthalmoplegia
Vitamin E deficiency
Anemic Year Old Child/Baby
Ataxia Disorder
Disease Mechanism & Classification
Class
CLASS/Pediatric disorders (ex)
Pathophysiology
Pathophysiology/Posterior columns cord def
Process
PROCESS/Deficiency (category)
PROCESS/Fat soluble vitamin deficiencies (ex)
Synonyms
Synonym
DEFIC VITAMIN E, Deficiencies Vitamin E, Deficiency of vitamin E, deficiency vitamin E, Hypovitaminosis E, Newborn, vitamin deficiency E, VITAMIN E DEFIC, Vitamin E Deficiencies, Vitamin E deficiency, Vitamin E deficiency (disorder), Vitamin E deficiency disease, Early Onset Ataxia/Children
Definition

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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External Links Related to Vitamin E defic. in newborns
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PubMed (National Library of Medicine)
NGC (National Guideline Clearinghouse)
Medscape (eMedicine)
Harrison's Online (accessmedicine)
NEJM (The New England Journal of Medicine)
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