Paradoxical weight loss with extra energy expenditure at brown adipose tissue in adolescent patients with Duchenne muscular dystrophy.
We examined the energy expenditure in patients with Duchenne muscular dystrophy(DMD) to evaluate the cause of the paradoxical weight loss observed in large numbers of adolescent patients before any obvious impairment of their swallowing function. In the morning, resting energy expenditure (REE)/m(2) was almost the same as that in normal controls despite a reduction in fat-free mass (FFM); thus, REE/m(2)/FFM was significantly increased in patients (median, 21.2 kcal/m(2)/FFM kg; range, 17.7 to 44.2, P =.012). A thermographic examination in the morning showed an obvious elevation of the body surface temperature on the back. This phenomenon was consistent with a paradoxical fall in the low frequency (LF)/high frequency (HF) ratio at night analyzed using the inter-RR spectrum by 24-hour electrocardiogram, which indicated relative activation of the sympathetic nervous system. The urinary secretion of norepinephrine at night was also significantly greater in patients (median, 0.119 microg/kg/h; range, 0.061 to 0.219, P =.011). These results suggest that paradoxical activation of the sympathetic nervous system may accelerate the production of heat in brown adipose tissue (BAT) and increase the level of energy consumption in patients, and that adolescent DMD patients may require greater caloric intake than expected to maintain body weight, which is important to improve the prognosis of their respiratory function.
Satomura
S
S
Department of Pediatrics, School of Medicine, University of Tokushima, Tokushima, Japan.
Yokota
I
I
Tatara
K
K
Naito
E
E
Ito
M
M
Kuroda
Y
Y
D002001:Adipose Tissue, Brown / Q000378:metabolism*; D000293:Adolescent; D000328:Adult; D001831:Body Temperature; D002395:Catecholamines / Q000652:urine; D004562:Electrocardiography; D004734:Energy Metabolism; D006801:Humans; D020388:Muscular Dystrophy, Duchenne / Q000378:metabolism / Q000503:physiopathology* / Q000652:urine; D013817:Thermography; D015431:Weight Loss
D002395:Catecholamines