Body composition and energy expenditure in Duchenne muscular dystrophy

Abstract

Objective: To investigate the relationship between resting energy expenditure (REE) and body composition in Duchenne Muscular Dystrophy (DMD). Design: An observational study. Setting: University Research Centre. Subjects: Nine Duchenne children (age range 6 -12 y), mean relative weight 128%, agreed to undergo the investigation and all of them completed the study; Interventions: Assessment of body composition (total body fat and skeletal muscle mass) by magnetic resonance imaging and resting energy expenditure by indirect calorimetry. Main outcome measures: Fat mass (FM; kg and percentage weight), fat-free mass (FFM; kg and percentage weight), muscle mass (kg and percentage weight), resting energy expenditure (kJ=kg body weight and kJ=kg fat-free mass). Results: In Duchenne children fat mass averages 32% and total skeletal muscle mass 20% of body weight. Resting energy expenditure per kg of body weight falls within the normal range for children of the same age range, while when expressed per kg of FFM is significantly higher than reference values. No relationship was found between REE and total skeletal muscle mass. Conclusions:Our results do not demonstrate a low REE in DMD boys; on the contrary REE per kg of FFM is higher than normal, probably due to the altered FFM composition. We suggest that the development of obesity in DMD children is not primarily due to a low REE but to other causes such as a reduction in physical activity and or overfeeding.

Gait energy expenditure in children with Duchenne muscular dystrophy: case study

“…Some authors report that, in these patients, the resting energy expenditure is lower than in the control group and emphasize the need for longitudinal studies, in order to understand these changes with the progression of the disease 10 . On the contrary, to other authors 12 , the resting energy expenditure is higher in patients with DMD than in healthy children, and yet, this expenditure is lower in obese rather than in nonobese patients with DMD. In this sense, it is emphasized that the analysis of energy expenditure should be related to the body composition analysis.…”

“…In these patients, the resting energy expenditure, measured by calorimetry, is the target of most studies [10][11][12][13] .…”

Energy expenditure analysis in Duchenne muscular dystrophy in land and aquatic environment

“…Além destes fatores, observamos a necessidade de incrementar o teste com comando verbal contínuo, a fim de estimular a deambulação durante os testes em solo e em água. Durante um estudo em solo foi observado que os pacientes com DMD obtiveram um gasto energéti-co menor do que as crianças saudáveis, porém, este fato aconteceu devido aos pacientes apresentarem um padrão muscular distrófico, fator este que favoreceu ao sedentarismo e obesidade 5 . Já em nosso estudo, analisamos o gasto energético nesta mesma população, mas em uma atividade dinâmica e em ambientes distintos: o aquático e terrestre.…”

“…Essas mudanças na composição corporal resultam em fraqueza muscular que afetam a funcionalidade e exigem uma demanda maior de energia. A perda da deambulação é uma das responsáveis por reduzir boa parte do gasto energético diário desses indivíduos [1][2][3][4][5] .…”

Neuromuscular Diseases and Rehabilitation

“…A person with a lower level of physical activity will later have an increased body weight even with a normal diet. Increased body weight makes the patient more inactive, and this continues in a vicious cycle [83][84][85]. Therefore, when it is considered that all of the components required for exercise are relatively affected in NMDs, a reduction in exercise capacity in this disease group is inevitable.…”