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.