Body composition and resting energy expenditure of individuals with duchenne and becker muscular dystrophy

摘要

Purpose: The relationship between body composition and resting energy expenditure (REE) was investigated in two boys and two men with Duchenne muscular dystrophy (DMD) (ages 11 to 22.4 years) and two boys with Becker muscular dystrophy (BMD) (ages 7.75 and 13.75 years). Methods: The REE was assessed by indirect calorimetry; body composition indices (weight, height, skinfolds, and mid-arm circumference) were measured using standardized techniques and compared with healthy reference data. Results: Those with DMD had reduced corrected mid-upper-arm muscle area (C-MUMA) in comparison with healthy peers, and approximately twice the subcutaneous fat levels of subjects with BMD and healthy peers. Boys with BMD had remarkably lower muscle status than did boys with DMD and healthy peers. In both groups, REE was lower than in healthy peers; REE was associated with body weight (r=0.85), height (r=0.92), mid-upper arm fat area (MUFA) (r=0.97), and C-MUMA (r=0.65). Conclusions: Individuals with muscular dystrophy (MD) exhibit considerable disease-specific alterations in body composition. The REE had a stronger relationship with growth (weight and height) and subcutaneous body fat composition and a weaker association with C-MUMA. Understanding the effect of MD on body composition and REE will allow dietitians to individualize energy recommendations.