title: Doubling diet fat on sugar ratio in children with mitochondrial OXPHOS disorders: Effects of a randomized trial on resting energy expenditure, diet induced thermogenesis and body composition.
abstract: Mitochondrial OXPHOS disorders (MODs) affect one or several complexes of respiratory chain oxidative phosphorylation. An increased fat/low-carbohydrate ratio of the diet was recommended for treating MODs without, however, evaluating its potential benefits through changes in the respective contributions of cell pathways (glycolysis, fatty acid oxidation) initiating energy production. Therefore, the objective of the present work was to compare Resting Energy Expenditure (REE) under basal diet (BD) and challenging diet (CD) in which fat on sugar content ratio was doubled. Diet-induced thermogenesis (DIT) and body compositions were also compared. Energetic vs regulatory aspects of increasing fat contribution to total nutritional energy input were essentially addressed through measures primarily aiming at modifying total fat amounts and not the types of fats in designed diets.
  
  
  
  In this randomized cross-over study, BD contained 10% proteins/30% lipids/60% carbohydrates (fat on sugar ratio = 0.5) and was the imposed diet at baseline. CD contained 10% proteins/45% lipids/45% carbohydrates (fat on sugar ratio = 1). Main and second evaluation criteria measured by indirect calorimetry (QUARK RMR®, Cosmed, Pavona; Italy) were REE and DIT, respectively. Thirty four MOD patients were included; 22 (mean age 13.2 ± 4.7 years, 50% female; BMI 16.9 ± 4.2 kg/m2) were evaluated for REE, and 12 (mean age 13.8 ± 4.8 years, 60% female; BMI 17.4 ± 4.6 kg/m2) also for DIT. OXPHOS complex deficiency repartition in 22 analysed patients was 55% for complex I, 9% for complex III, 27% for complex IV and 9% for other proteins.
  
  
  
  Neither carry-over nor period effects were detected (p = 0.878; ANOVA for repeated measures). REE was similar between BD vs CD (1148.8 ± 301.7 vs 1156.1 ± 278.8 kcal/day; p = 0.942) as well as DIT (peak DIT 260 vs 265 kcal/day; p = 0.842) and body composition (21.9 ± 13.0 vs 21.6 ± 13.3% of fat mass; p = 0.810).
  
  
  
  Doubling diet fat on sugar ratio does not appear to improve, per se, energetic status and body composition of patients with MODs.
authors.affiliation: Centre d'Investigation Clinique, CIC-1403-Inserm-CH&U, Lille University Hospital, F-59000 Lille, France; LIRIC- Lille Inflammation Research International Center/UMR U995 Inserm, Lille, France. Electronic address: laurent.beghin@chru-lille.fr.
authors.forename: Laurent
authors.initials: L
authors.lastname: Béghin
authors.affiliation: Centre d'Investigation Clinique, CIC-1403-Inserm-CH&U, Lille University Hospital, F-59000 Lille, France. Electronic address: Stephanie.coopman@chru-lille.fr.
authors.forename: Stéphanie
authors.initials: S
authors.lastname: Coopman
authors.affiliation: Reference Center for Inherited Metabolic Diseases, Robert Debré University Hospital, Paris, France. Electronic address: manuel.schiff@aphp.fr.
authors.forename: Manuel
authors.initials: M
authors.lastname: Schiff
authors.affiliation: Inserm, Department of Biochemistry and Molecular Biology, HMNO, CBP, CHRU Lille and RADEME EA 7364, Lille Nord of France University, F-59000, Lille, France. Electronic address: joseph.vamecq@inserm.fr.
authors.forename: Joseph
authors.initials: J
authors.lastname: Vamecq
authors.affiliation: Reference Center for Inherited Metabolic Diseases in Child and Adulthood, Lille University Children's Hospital Jeanne de Flandre, and RADEME EA 7364, Lille University, F-59000 Lille, France. Electronic address: karine.mention@chru-lille.fr.
authors.forename: Karine
authors.initials: K
authors.lastname: Mention-Mulliez
authors.affiliation: Inserm U 1069, F Rabelais University, Tours, F-37000, France. Electronic address: regis.hankard@univ-tours.fr.
authors.forename: Régis
authors.initials: R
authors.lastname: Hankard
authors.affiliation: Pediatric Neurology Unit, Lille University Hospital, F-59000, Lille, France.
authors.forename: Jean-Marie
authors.initials: JM
authors.lastname: Cuisset
authors.affiliation: Reference Center for Inherited Metabolic Diseases, Robert Debré University Hospital, Paris, France.
authors.forename: Hélène
authors.initials: H
authors.lastname: Ogier
authors.affiliation: Centre d'Investigation Clinique, CIC-1403-Inserm-CH&U, Lille University Hospital, F-59000 Lille, France; LIRIC- Lille Inflammation Research International Center/UMR U995 Inserm, Lille, France. Electronic address: Frederic.gottrand@chru-lille.fr.
authors.forename: Frédéric
authors.initials: F
authors.lastname: Gottrand
authors.affiliation: Reference Center for Inherited Metabolic Diseases in Child and Adulthood, Lille University Children's Hospital Jeanne de Flandre, and RADEME EA 7364, Lille University, F-59000 Lille, France. Electronic address: dries.dobbelaere@chru-lille.fr.
authors.forename: Dries
authors.initials: D
authors.lastname: Dobbelaere
mesh_terms: D000293:Adolescent; D001481:Basal Metabolism; D001823:Body Composition; D002153:Calorimetry, Indirect; D002648:Child; D002675:Child, Preschool; D018592:Cross-Over Studies; D004032:Diet; D004040:Dietary Carbohydrates / Q000008:administration & dosage; D004041:Dietary Fats / Q000008:administration & dosage*; D004044:Dietary Proteins / Q000008:administration & dosage; D000073417:Dietary Sugars / Q000008:administration & dosage; D004734:Energy Metabolism; D005260:Female; D006801:Humans; D008297:Male; D028361:Mitochondrial Diseases / Q000378:metabolism*; D011446:Prospective Studies; D022722:Thermogenesis; D055815:Young Adult
chemical_list: D004040:Dietary Carbohydrates; D004041:Dietary Fats; D004044:Dietary Proteins; D000073417:Dietary Sugars
keywords: Body composition; Diet induced thermogenesis; Energy expenditure; Mitochondrial disorders or diseases