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J Lipid Res. 2018 Sep;59(9):1640-1648. doi: 10.1194/jlr.M085043. Epub 2018 Jul 18.

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

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Biochemistry Department, Lyon Sud Hospital, Hospices Civils de Lyon, Lyon, France.
INSERM U1060, INRA UMR 1397, INSA-Lyon, CarMeN Laboratory, Université Lyon 1, Lyon, France.
Calvagone, Liergues, France.
Pediatric Hepato-Gastroenterology and Nutrition Unit, Hôpital Femme Mère Enfant de Lyon, Dyslipidemia Unity Hospices Civils de Lyon, Lyon, Bron, France.
Department of Biochemistry and Molecular Biology, Centre de Biologie et de Pathologie Est, Hospices Civils de Lyon, Lyon, Bron, France.
Fédération d'Endocrinologie, Maladies Métaboliques, Diabète et Nutrition, Hôpital Louis Pradel, Hospices Civils de Lyon, Lyon, Bron, France.
INRA, INSERM, Aix-Marseille University, Marseille, France.
Research Centre, CHU Sainte-Justine, and Department of Nutrition, Université de Montréal, Montréal, Québec, Canada.
INSERM U1060, INRA UMR 1397, INSA-Lyon, CarMeN Laboratory, Université Lyon 1, Lyon, France


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 different by formulation type in ABL or CMRD. This study provides new insights about vitamin E status in ABL and CMRD and suggests the potential of different formulations as treatment options.


Anderson disease; absorption; adipose tissue; hypocholesterolemia; lipid and lipoprotein metabolism; metabolic disease; tocopherol

[Available on 2019-09-01]

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