Efficacy of two vitamin E formulations in patients with abetalipoproteinemia and chylomicron retention disease.

Journal of Lipid Research
Charlotte CuerqNoel Peretti

Abstract

Abetalipoproteinemia (ABL) and chylomicron retention disease (CMRD) are extremely rare recessive forms of hypobetalipoproteinemia characterized by intestinal lipid malabsorption and severe vitamin E deficiency. Vitamin E is often supplemented in the form of fat-soluble vitamin E acetate, but fat malabsorption considerably limits correction of the deficiency. In this crossover study, we administered two different forms of vitamin E, tocofersolan (a water-soluble derivative of RRR-α-tocopherol) and α-tocopherol acetate, to three patients with ABL and four patients with CMRD. The aims of this study were to evaluate the intestinal absorption characteristics of tocofersolan versus α-tocopherol acetate by measuring the plasma concentrations of α-tocopherol over time after a single oral load and to compare efficacy by evaluating the ability of each formulation to restore vitamin E storage after 4 months of treatment. In patients with ABL, tocofersolan and α-tocopherol acetate bioavailabilities were extremely low (2.8% and 3.1%, respectively). In contrast, bioavailabilities were higher in patients with CMRD (tocofersolan, 24.7%; α-tocopherol acetate, 11.4%). Plasma concentrations of α-tocopherol at 4 months were not significantly diffe...Continue Reading

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Citations

Jan 15, 2019·Current Opinion in Lipidology·Emile LevySchohraya Spahis
Mar 31, 2019·Nature Communications·Cyrielle CaussyRohit Loomba
Sep 10, 2019·The American Journal of Clinical Nutrition·Emmanuelle Reboul
Apr 15, 2021·Journal of Pediatric Gastroenterology and Nutrition·Samira SissaouiNoël Peretti

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