Vitamin E deficiency and neurologic disease in childrenwith cholestasis: A prospective study

Guggenheim, Mary Anne; Jackson, Virginia; Lilly, John R.; Silverman, Arnold

doi:10.1016/s0022-3476(83)80189-9

Cited by 53 publications

(9 citation statements)

References 12 publications

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“…The primary, and most serious, clinical manifestation of human vitamin E deficiency is a peripheral neuropathy with degeneration of large-caliber axons in sensory neurons [143-146]. The development of a progressive ataxia appears more rapidly in infants and young children with malabsorption from birth than in adults, suggesting that the developing nervous system is dependent on adequate vitamin E for normal development [147].…”

Section: Vitamin Ementioning

confidence: 99%

Vitamins C and E: Beneficial effects from a mechanistic perspective

Traber

Stevens

2011

Free Radical Biology and Medicine

788

550

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The mechanistic properties of two dietary antioxidants that are required by humans, vitamins C and E, are discussed relative to their biological effects. Vitamin C (ascorbic acid) is an essential cofactor for α-ketoglutarate-dependent dioxygenases. Examples are prolyl hydroxylases, which play a role in the biosynthesis of collagen and in down-regulation of hypoxia-inducible factor (HIF)-1, a transcription factor that regulates many genes responsible for tumor growth, energy metabolism, and neutrophil function and apoptosis. Vitamin C-dependent inhibition of the HIF pathway may provide alternative or additional approaches for controlling tumor progression, infections and inflammation. Vitamin E (α-tocopherol) functions as an essential lipid soluble antioxidant, scavenging hydroperoxyl radicals in lipid milieu. Human symptoms of vitamin E deficiency suggest that its antioxidant properties play a major role in protecting erythrocyte membranes and nervous tissues. As an antioxidant, vitamin C provides protection against oxidative stress-induced cellular damage by scavenging of reactive oxygen species, vitamin E-dependent neutralization of lipid hydroperoxyl radicals, and by protecting proteins from alkylation by electrophilic lipid peroxidation products. These bioactivities bear relevance to inflammatory disorders. Vitamin C plays also a role in the function of endothelial nitric oxide synthase (eNOS) by recycling the eNOS cofactor, tetrahydrobiopterin, which is relevant to arterial elasticity and blood pressure regulation. Evidence from plants supports a role for vitamin C in the formation of covalent adducts with electrophilic secondary metabolites. Mechanism-based effects of vitamin C and E supplementation on biomarkers and on clinical outcomes from randomized, placebo-controlled trials are emphasized in this review.

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Section: Vitamin Ementioning

confidence: 99%

Vitamins C and E: Beneficial effects from a mechanistic perspective

Traber

Stevens

2011

Free Radical Biology and Medicine

788

550

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“…A number of reports have now appeared relating a neurological syndrome to severe vitamin E deficiency in patients with a variety of chronic fat malabsorptive states. The majority ofthese reports have been in children with cholestatic liver diseases (Rosenblum et al, 1981;Elias et al, 1981;Guggenheim et al, 1982Guggenheim et al, , 1983Alvarez et al, 1983;Sokol et al, 1983). The neurological features in abetalipoproteinaemia and in these patients with cholestasis are very similar (Muller et al, 1983) although it initially appeared that retinal changes were less common in the latter group.…”

Section: Other Chronic Disorders Of Fat Absorptionmentioning

confidence: 99%

Vitamin E—its role in neurological function

Muller

1986

Postgraduate Medical Journal

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Summary:Studies in patients with abetalipoproteinaemia, other chronic and severe fat malabsorptive states and a selective defect in vitamin E absorption, together with neuropathological studies in the vitamin E deficient human, monkey and rat indicate that vitamin E is important for normal neurological function. Appropriate vitamin E supplementation is, therefore, advisable for all patients with chronic fat malabsorption who have low serum vitamin E concentrations. Serum vitamin E concentrations should also be measured in patients with spinocerebellar disorders, whatever the aetiology.

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“…The utility of breath ethane or pentane analysis for the diagnosis of vitamin E deficiency in pediatric patients has not been explored. Vitamin E deficiency, common in children with chronic severe liver disease, may cause progressive neurologic abnormalities unless treated (6,7). Massive oral doses (1 00-200 IU/kg of water miscible vitamin E) may not correct the deficiency in this population, and parenteral vitamin E therapy may be necessary (8).…”

mentioning

confidence: 99%

Utility of Breath Ethane as a Noninvasive Biomarker of Vitamin E Status in Children

et al. 1991

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ABSTRACT. The purpose of our study was to determine if the ethane content of expired air could be a useful index of vitamin E status in children. Eight children with vitamin E deficiency secondary to chronic severe liver disease were studied: six of these children were treated with parenteral vitamin E (2-5 mg/kg/dose every 4-7 d). Measures of vitamin E status pre-and posttherapy were: serum vitamin

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Vitamin E deficiency and neurologic disease in childrenwith cholestasis: A prospective study

Cited by 53 publications

References 12 publications

Vitamins C and E: Beneficial effects from a mechanistic perspective

Vitamins C and E: Beneficial effects from a mechanistic perspective

Vitamin E—its role in neurological function

Utility of Breath Ethane as a Noninvasive Biomarker of Vitamin E Status in Children

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