Multicenter trial of d-α-tocopheryl polyethylene glycol 1000 succinate for treatment of vitamin E deficiency in children with chronic cholestasis

Sokol, Ronald J.; Butler–Simon, Nancy; Conner, Colin; Heubi, James E.; Sinatra, Frank; Suchy, Frederick J.; Heyman, Melvin B.; Perrault, Jean; Rothbaum, Robert; Levy, Joseph; Iannaccone, Susan T.; Shneider, Benjamin L.; Koch, Thomas; Narkewicz, Michael R.

doi:10.1016/0016-5085(93)90652-s

Cited by 123 publications

(67 citation statements)

References 42 publications

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“…Low levels despite supplementation [106] may be due to lack of adherence, malabsorption despite pancreatic enzyme supplementation, intestinal resection, or liver disease. Individuals with cholestatic liver disease should be supplemented with D-α-tocopheryl-polyethylene glycol 1000 succinate (TPGS), the only truly water-soluble form available in the United States [111].…”

Section: Vitamin E (α-Tocopherol)mentioning

confidence: 99%

Antioxidants in cystic fibrosis☆Conclusions from the CF Antioxidant Workshop, Bethesda, Maryland, November 11-12, 2003

Cantin

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Cross

et al. 2007

Free Radical Biology and Medicine

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Although great strides are being made in the care of individuals with cystic fibrosis (CF), this condition remains the most common fatal hereditary disease in North America. Numerous links exist between progression of CF lung disease and oxidative stress. The defect in CF is the loss of function of the transmembrane conductance regulator (CFTR) protein; recent evidence that CFTR expression and function are modulated by oxidative stress suggests that the loss may result in a poor adaptive response to oxidants. Pancreatic insufficiency in CF also increases susceptibility to deficiencies in lipophilic antioxidants. Finally the airway infection and inflammatory processes in the CF lung are potential sources of oxidants that can affect normal airway physiology and contribute to the mechanisms causing characteristic changes associated with bronchiectasis and loss of lung function. These multiple abnormalities in the oxidant/antioxidant balance raise several possibilities for therapeutic interventions that must be carefully assessed. IntroductionCystic fibrosis (CF) is the most common fatal disease caused by a single gene defect in Europe and North America [1,2]. The defective gene was identified in 1989 [3][4][5] and shown to encode the cystic fibrosis trans-membrane conductance regulator protein (CFTR). CFTR is a cyclic AMP-regulated anion channel with greatest selectivity for chloride, and bicarbonate [6][7][8]. Furthermore, CFTR regulates sodium transport across epithelial membranes through interactions with the epithelial sodium channel ENaC [9], and facilitates the trans-membrane export of the small organic anionic peptide glutathione [10]. The expression of CFTR is located in the apical membrane of epithelial cells that line mucous membranes and submucosal glands ☆ A list of participants may be found in the Acknowledgments.

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Section: Vitamin E (α-Tocopherol)mentioning

confidence: 99%

Antioxidants in cystic fibrosis☆Conclusions from the CF Antioxidant Workshop, Bethesda, Maryland, November 11-12, 2003

Cantin

White

Cross

et al. 2007

Free Radical Biology and Medicine

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“…Oral d-alpha tocopheryl polyethylene glycol-1000 succinate (TPGS), a water-soluble form of vitamin E, is absorbed well in vitamin E-deficient children with chronic cholestasis who fail to absorb other forms of vitamin E. 19,20 Additionally, TPGS, when given concurrently with other lipid-soluble compounds (such as cyclosporine 21 ), increases their absorption. This effect of TPGS is related to its amphipathic structure, resulting in the formation of micelles in the absence of bile acids.…”

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confidence: 99%

Comparison of indices of vitamin A status in children with chronic liver disease

et al. 2005

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Malabsorption of fat-soluble vitamins is a major complication of chronic cholestatic liver disease. The most accurate way to assess vitamin A status in children who have cholestasis is unknown. The goal of this study was to assess the accuracy of noninvasive tests to detect vitamin A deficiency. Children with chronic cholestatic liver disease (n ‫؍‬ 23) and noncholestatic liver disease (n ‫؍‬ 10) were studied. Ten cholestatic patients were identified as vitamin A-deficient based on the relative dose response (RDR). Compared with the RDR, the sensitivity and specificity to detect vitamin A deficiency for each test was, respectively: serum retinol, 90% and 78%; retinolbinding protein (RBP), 40% and 91%; retinol/RBP molar ratio, 60% and 74%; conjunctival impression cytology, 44% and 48%; slit-lamp examination, 20% and 66%; tear film break-up time, 40% and 69%; and Schirmer's test, 20% and 78%. We developed a modified oral RDR via oral coadministration of d-alpha tocopheryl polyethylene glycol-1000 succinate and retinyl palmitate. This test had a sensitivity of 80% and a specificity of 100% to detect vitamin A deficiency. In conclusion, vitamin A deficiency is relatively common in children who have chronic cholestatic liver disease. Our data suggest that serum retinol level as an initial screen followed by confirmation with a modified oral RDR test is the most effective means of identifying vitamin A deficiency in these subjects. (HEPATOLOGY 2005;42:782-792.)

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“…Similar results have been reported by others (Azizi et al 1978;Kane and Havel 2001). Vitamin E therapy also halted or reversed the progression of neurological abnormalities in patients with chronic cholestatic liver disease (Alvarez et al 1983;Guggenheim et al 1982;Perlmutter et al 1987;Sokol et al 1985Sokol et al , 1993 The electrophysiological results in group RϪ were, as expected, very similar to those recorded in the deficient animals (group D). After repletion with vitamin E, with the exception of the VEP amplitudes, group Rϩ electrophysiological parameters tended to diverge from group D and become more like group C (controls).…”

Section: Discussionmentioning

confidence: 62%

“…The effects of repleting vitamin E-deficient human subjects with the vitamin have been studied in patients with abetalipoproteinemia (Azizi et al 1978;Bishara et al 1982;Brin et al 1986;Fagan and Taylor 1987;Muller et al 1977Muller et al , 1983, chronic cholestasis (Alvarez et al 1983;Guggenheim et al 1982;Perlmutter et al 1987;Sokol et al 1985Sokol et al , 1993, cystic fibrosis (Elias et al 1981), multiple ileal resection (Harding et al 1982;Howard et al 1982), and ataxia with vitamin E deficiency (AVED) (Harding et al 1985;Sokol et al 1988). The progression of the neurological changes associated with these disorders has been halted or, in some cases, reversed after appropriate vitamin E therapy.…”

Section: Introductionmentioning

confidence: 99%

Effects on Neural Function of Repleting Vitamin E–Deficient Rats With α-Tocopherol

Hayton¹,

Kriss²,

Wade³

et al. 2006

Journal of Neurophysiology

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Multicenter trial of d-α-tocopheryl polyethylene glycol 1000 succinate for treatment of vitamin E deficiency in children with chronic cholestasis

Cited by 123 publications

References 42 publications

Antioxidants in cystic fibrosis☆Conclusions from the CF Antioxidant Workshop, Bethesda, Maryland, November 11-12, 2003

Antioxidants in cystic fibrosis☆Conclusions from the CF Antioxidant Workshop, Bethesda, Maryland, November 11-12, 2003

Comparison of indices of vitamin A status in children with chronic liver disease

Effects on Neural Function of Repleting Vitamin E–Deficient Rats With α-Tocopherol

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