Frequency and Clinical Progression of the Vitamin E Deficiency Neurologic Disorder in Children With Prolonged Neonatal Cholestasis

Sokol, Ronald J.; Guggenheim, Mary Anne; Heubi, James E.; Iannaccone, Susan T.; Butler–Simon, Nancy; Jackson, Virginia; Miller, Christopher; Cheney, M. C.; Balistreri, William F.; Silverman, Arnold

doi:10.1001/archpedi.1985.02140140045024

Cited by 23 publications

(23 citation statements)

References 22 publications

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“…This potentiating effect of vitamin E deficiency on copper toxicity may have clinical relevance in regard to chronic cholestatic liver diseases, where both secondary hepatic copper overload (37,38) and vitamin E deficiency (39,40) are common. Although it is not certain if copper overload during cholestasis is toxic to the liver (41), we have observed that reversing vitamin E deficiency in children with chronic cholestasis and secondary hepatic copper overload was associated with a decrease of fasting serum bile acid concentrations (42), suggesting that vitamin E repletion lessened hepatic injury and improved hepatic function.…”

Section: Discussionmentioning

confidence: 95%

Copper Toxicity and Lipid Peroxidation in Isolated Rat Hepatocytes: Effect of Vitamin E

et al. 1989

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ABSTRACT. We investigated the role of lipid peroxidation as the mechanism mediating copper toxicity in isolated rat hepatocytes and the modulating effect of vitamin E. Hepatocytes, isolated from rats fed diets containing deficient (E-), sufficient (E+), and excess (E++) amounts of vitamin E, were incubated with CuClz (0-2400 pM) for 150 min. Dose and time-dependent decreases in hepatocyte viability (determined by trypan blue exclusion and lactate dehydrogenase release) due to copper toxicity correlated with production of malonyldialdehyde in E-and E+ hepatocytes. However, malonyldialdehyde generation did not accompany copper toxicity in E++ cells. Copper toxicity was enhanced in E-compared to E+ and E++ hepatocytes as assessed by cell viability studies and ultrastructural plasma membrane bleb formation.

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Section: Discussionmentioning

confidence: 95%

Copper Toxicity and Lipid Peroxidation in Isolated Rat Hepatocytes: Effect of Vitamin E

et al. 1989

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“…Although lower adipose tissue a-tocopherol concentrations have been reported in vitamin E-deficient children, these are usually observed after years of inadequate vitamin E absorption, such as in children with cholestatic liver disease (19) or in those with a genetic defect in lipoprotein synthesis, eg, abetalipoproteinemia (17). Moreover, it is unlikely that the depletion in adipose tissue vitamin E is due to dietary changes because Handelman et al (23) studied adipose tissue tocopherol concentrations in adults before, during supplementation with 800 IU/d for 1 y, and after discontinuation of supplementation for 1 y.…”

Section: Discussionmentioning

confidence: 99%

“…The next step is to consider what effect additional vitamin E supplementation may have in ameliorating the oxidative stress of burn injury in patients. Vitamin E deficiency is manifested by peripheral neuropathy in noninjured patients (19,20). In burn patients, peripheral neuropathy is common (21) and has many possible etiologies.…”

Section: Discussionmentioning

confidence: 99%

“…Moreover, adipose tissue a-tocopherol concentrations have been used to demonstrate the adequacy of vitamin E supplementation in previously vitamin E-deficient subjects (17), and adipose tissue a-tocopherol concentrations have been shown to be predictive of peripheral nerve concentrations in vitamin E-deficient humans (18). This finding is import because vitamin E deficiency in humans causes peripheral neuropathy due to a dying back of large caliber fibers in sensory neurons (19,20). Remarkably, peripheral neuropathy in burn patients is also common (21), but the possible etiologies are many.…”

Section: Introductionmentioning

confidence: 99%

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α-Tocopherol adipose tissue stores are depleted after burn injury in pediatric patients

Traber¹,

Leonard²,

Traber³

et al. 2010

The American Journal of Clinical Nutrition

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Background: We previously showed that thermal injury depletes plasma vitamin E in pediatric burn patients; however, plasma changes may reflect immediate alterations in vitamin E nutriture. Adipose tissue a-tocopherol concentrations are generally accepted to reflect long-term vitamin E status. Objective: To test the hypothesis that thermal injury depletes body stores of vitamin E, a-tocopherol concentrations were measured in adipose tissue samples. Design: Pediatric patients (n = 8) were followed up to 1 y after burn injury. Surgically obtained samples were collected at various intervals and stored at 280°C in a biorepository. a-and c-Tocopherols, cholesterol, and triglycerides were measured in the same tissue aliquot. Results: During the first week after injury, adipose tissue a-tocopherol concentrations were within the expected normal range of 199 6 40 nmol/g adipose tissue but were substantially lower at weeks 2 and 3 (133 6 13 and 109 6 8 nmol/g adipose tissue, respectively). Individual rates of decrease, estimated by linear regression, showed that adipose tissue a-tocopherol decreased by an average of 6.1 6 0.6 nmol/g daily. During the first month after injury, adipose tissue triglyceride concentrations also decreased, whereas no changes in cholesterol concentrations occurred. Conclusions: These data emphasize that the burn injury experienced by these pediatric patients altered their metabolism such that vitamin E status diminished during the month after injury. Further studies are needed to evaluate the mechanism and consequences of the observed vitamin E depletion. This trial was registered at clinicaltrials.gov as NCT00675714.Am J Clin Nutr 2010;92:1378-84.

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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).…”

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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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Frequency and Clinical Progression of the Vitamin E Deficiency Neurologic Disorder in Children With Prolonged Neonatal Cholestasis

Cited by 23 publications

References 22 publications

Copper Toxicity and Lipid Peroxidation in Isolated Rat Hepatocytes: Effect of Vitamin E

Copper Toxicity and Lipid Peroxidation in Isolated Rat Hepatocytes: Effect of Vitamin E

α-Tocopherol adipose tissue stores are depleted after burn injury in pediatric patients

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

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