Resting Energy Expenditure and Energy Substrate Utilization in Children with Duchenne Muscular Dystrophy

Abstract

The high prevalence of obesity at an early stage of Duchenne muscular dystrophy (DMD) could result not solely from reduced physical activity, but may also involve low resting energy expenditure (REE), abnormal nutrient utilization, or overfeeding. We hypothesized that the dramatic muscle mass loss in DMD should reduce the REE. REE was measured by indirect calorimetry in 13 9-13-y-old DMD boys (5 obese, 8 nonobese) and 9 male age-matched controls. Muscle mass was estimated from 3-d creatinine excretion in urine. Daily energy intake was estimated from 7-d diet records. In the nonobese DMD group (NODMD) the muscle mass was reduced by 71%, and the REE was 13% lower than in controls (47.5 versus 54.6 kcal.h-1, p < 0.05). Postabsorptive respiratory quotients appeared higher in both DMD groups than in the controls; however, the difference was significant only for the NODMD group (0.88 versus 0.83, p < 0.05). Respiratory quotients were not different between the two DMD groups. Diet records were not contributive in revealing a different dietary behavior between groups. Our results suggest that: 1) muscle mass loss in DMD is associated with a low REE, 2) low postabsorptive fat utilization might occur at an early stage of the disease, and 3) obesity is not associated with an increase in fat utilization in DMD. This study warrants further research to test low REE and low fat utilization as risk factors in developing obesity in DMD.

Body composition and energy expenditure in Duchenne muscular dystrophy

“…When the group was divided according to body weight, obese children showed a lower REE than normal-weight ones. This result has already been reported by Hankard et al (1996) on Duchenne boys and by Maffeis et al (1991) on healthy children.…”

“…Likewise, Hankard (1996) observed a significantly higher REE (kJ=kg FFM) in obese Duchenne children than controls.…”

“…Previous studies quantitatively estimated muscle mass from creatinine excretion or total body potassium (Hankard et al, 1996;Griggs et al, 1983). To our knowledge, just one preliminary study described an MRI quantitative approach to quantify the volume of whole-body muscle in four adult muscular dystrophy patients (Gong et al, 2000) and found a reduction of 27% of muscle volume compared with control subjects.…”

“…Hankard et al, (1996) first hypothesized that muscle mass loss would decrease REE and therefore contribute to the onset of obesity. In their study, a 71% muscle loss was estimated by creatinine excretion in a group of non-obese DMD boys aged 10 and it was associated with a 13% decrease in REE (47.5 vs 54.6 kcal=h, P < 0.05).…”

Avaliação dos efeitos da suplementação com ácidos ascórbicos sobre os aspectos morfoquantitativos do plexo mioentérico do jejuno de camundongos mdx com ausência de distrofina

“…Autores como Hankard et al (1996) formulam uma outra hipótese para o aumento de peso de animais mdx, associando essa situação com a redução do gasto de energia devido, principalmente, à perda da massa muscular, uma vez que o músculo esquelético é a principal fonte de consumo de energia (HANKARD et al, 1996).…”