Efficacy of two vitamin E formulations in patients with abetalipoproteinemia and chylomicron retention disease
| Autor: | Philippe Moulin, Jocelyne Drai, Emmanuelle Reboul, Emile Levy, Charlotte Cuerq, Lioara Restier, Noël Peretti, Mathilde Di Filippo, Marie-Caroline Michalski, Alain Lachaux, Emilie Blond, Pierre Poinsot, Agnès Sassolas, Sybil Charrière, Cyrielle Caussy, Christian Laveille, Christophe Marçais, Emilie Henin |
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| Přispěvatelé: | Cardiovasculaire, métabolisme, diabétologie et nutrition (CarMeN), Institut National de la Recherche Agronomique (INRA)-Université Claude Bernard Lyon 1 (UCBL), Université de Lyon-Université de Lyon-Institut National des Sciences Appliquées de Lyon (INSA Lyon), Université de Lyon-Institut National des Sciences Appliquées (INSA)-Institut National des Sciences Appliquées (INSA)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Hospices Civils de Lyon (HCL), Laboratoire de Mécanique des Systèmes et des Procédés (LMSP), Centre National de la Recherche Scientifique (CNRS), Centre Hospitalier Lyon Sud [CHU - HCL] (CHLS), Hospices Civils de Lyon (HCL), Nutrition, obésité et risque thrombotique (NORT), Aix Marseille Université (AMU)-Institut National de la Recherche Agronomique (INRA)-Institut National de la Santé et de la Recherche Médicale (INSERM), Centre recherche en CardioVasculaire et Nutrition = Center for CardioVascular and Nutrition research (C2VN), Institut National de la Recherche Agronomique (INRA)-Aix Marseille Université (AMU)-Institut National de la Santé et de la Recherche Médicale (INSERM), Université Claude Bernard Lyon 1 (UCBL), Université de Lyon, Université de Montréal (UdeM), Centre recherche en CardioVasculaire et Nutrition (C2VN), ProdInra, Migration |
| Rok vydání: | 2018 |
| Předmět: |
Male
0301 basic medicine apoprotein-b [SDV]Life Sciences [q-bio] medicine.medical_treatment tissu adipeux métabolisme des lipides Anderson disease adipose-tissue Biochemistry Gastroenterology Hypobetalipoproteinemias chemistry.chemical_compound 0302 clinical medicine Endocrinology liquid-chromatography Vitamin E chronic cholestasis triglyceride transfer protein Research Articles Vitamin E Acetate lipoprotéine [SDV.MHEP] Life Sciences [q-bio]/Human health and pathology chylomicron Middle Aged tocopherol metabolic disease Abetalipoproteinemia adipose tissue 3. Good health lipid and lipoprotein metabolism absorption hypocholesterolemia Female 030211 gastroenterology & hepatology Vitamin E deficiency Safety Chylomicron retention disease Adult Biochemistry & Molecular Biology medicine.medical_specialty Drug Compounding Drug Storage Médecine humaine et pathologie Biological Availability andersons disease QD415-436 Tocofersolan 03 medical and health sciences Malabsorption Syndromes alpha-tocopherol Internal medicine abetalipoproteinémie medicine Humans hypocholestérolemie adipose business.industry tissue Cell Biology fatty-acids maladie métabolique mutations medicine.disease 030104 developmental biology Fat-Soluble Vitamin Intestinal Absorption chemistry Case-Control Studies Human health and pathology business alpha-Tocopherol [SDV.MHEP]Life Sciences [q-bio]/Human health and pathology lipid-metabolism |
| Zdroj: | Journal of Lipid Research Journal of Lipid Research, American Society for Biochemistry and Molecular Biology, 2018, 59 (9), pp.1640--1648 Journal of Lipid Research, 2018, 59 (9), pp.1640-1648. ⟨10.1194/jlr.M085043⟩ Journal of Lipid Research, American Society for Biochemistry and Molecular Biology, 2018, 59 (9), pp.1640-1648. ⟨10.1194/jlr.M085043⟩ Journal of Lipid Research, Vol 59, Iss 9, Pp 1640-1648 (2018) Journal of Lipid Research, 2018, 59 (9), pp.1640--1648 Journal of Lipid Research 9 (59), 1640-1648. (2018) |
| ISSN: | 0022-2275 |
| DOI: | 10.1194/jlr.m085043 |
| Popis: | International audience; 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-alpha-tocopherol) and alpha-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 alpha-tocopherol acetate by measuring the plasma concentrations of alpha-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 alpha-tocopherol acetate bioavailabilities were extremely low (2.8% and 3.1%, respectively). In contrast, bioavailabilities were higher in patients with CMRD (tocofersolan, 24.7%; alpha-tocopherol acetate, 11.4%). Plasma concentrations of alpha-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. |
| Databáze: | OpenAIRE |
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