Perfil nutricional e metabolismo de repouso de crianças e adolescentes com distrofia muscular de Duchenne

Abstract

Duchenne muscular dystrophy (DMD) is a progressive disease, X-linked, caused by mutations in the dystrophin gene, which leads to lack of expression of this protein in the muscles, resulting in progressive muscle weakness. Corticosteroid treatment is accepted as standard of care in patients until loss of ambulation, however, side effects such as weight gain need continuous attention. DMD patients are at risk of developing nutritional disorders that can interfere with disease progression.This study was conducted with the objective of drawing the nutritional profile and assess the resting metabolic rate (RMR) of childrens and adolescents with DMD followed at HC / UFMG. We conducted a cross-sectional study with 35 boys aged 5 to 17 years. We analyzed stature, nutritional status according to body mass index (BMI) by the curves of WHO and specific curves for DMD, and ideal weight curve (IW) for DMD. Body composition was assessed by skinfold thickness, and arm muscle area. RMR was measured by indirect calorimetry and compared with energy expenditure (EE) estimated by formulas. We also evaluated caloric intake and investigated the lipid profile, glucose and serum 25-hydroxyvitamin D (Vit. D). By WHO curves, 19,3% of the patients were classified as underweight (UW) and 38.7% as overweight (OW). In the specific curves for DMD, the BMI classification was different from the WHO in 26.7% of boys. By IW curves for the disease, 9.7% of children had UW and 71% OW. Short stature was observed in 20% of subjects. Muscle depletion was identified in 63.7% of boys and 72.7% had excessive body fat mass. The RMR average was 982.65 ± 247.23 kcal, significantly lower than the estimated values of EE. The average caloric intake was 1292.20 ± 245.76 kcal, significantly higher than the RMR. The RMR/weight ratio was lower in children who did not walk. Patients had blood glucose and LDL cholesterol at acceptable levels, triglycerides and HDL in the borderline range and serum Vit. D deficient. We concluded that patients with DMD present nutritional disorders. Excessive weight (overweight and obesity) is more frequent. Among the factors that may be related to this finding, mentioning to low RMR, low physical activity and calorie intake higher than RMR. Meauring RMR at different stages of DMD allows better matching of caloric recommendation, contributing to an individualized nutritional control, avoiding nutritional disorders. The use of specific curves for the disease can also be an important factor in monitoring these children.