Increased resting energy expenditure in subjects with Emery-Dreifuss muscular dystrophy

• Published in: Neuromuscular disorders : NMD Vol. 14; no. 2; pp. 142 - 146
• Main Authors: Vaisman, N, Katzenellenbogen, S, Nevo, Y
• Format: Journal Article
• Language: English
• Published: England Elsevier B.V 01.02.2004
• Subjects: Absorptiometry, Photon; Adipose Tissue - metabolism; Adipose Tissue - pathology; Adipose Tissue - physiopathology; Adult; Atrophy - metabolism; Atrophy - pathology; Atrophy - physiopathology; Body Mass Index; Creatinine - urine; Emery-Dreifuss muscular dystrophy; Energy Metabolism - genetics; Humans; Hypercalcemia - metabolism; Hypercalcemia - physiopathology; Hyperkalemia - metabolism; Hyperkalemia - physiopathology; Lean body mass; Longitudinal Studies; Male; Muscle, Skeletal - metabolism; Muscle, Skeletal - pathology; Muscle, Skeletal - physiopathology; Muscular Dystrophy, Emery-Dreifuss - metabolism; Muscular Dystrophy, Emery-Dreifuss - pathology; Muscular Dystrophy, Emery-Dreifuss - physiopathology; Reference Values; Rest - physiology; Resting energy expenditure; Up-Regulation - genetics; Lean body mass; Emery-Dreifuss muscular dystrophy; Resting energy expenditure
• ISSN: 0960-8966; 1873-2364
• DOI: 10.1016/j.nmd.2003.10.012

We have studied changes in energy expenditure and body composition in adult males with Emery-Dreifuss muscular dystrophy, age-matched males with hyperCKemia and age-matched healthy controls. All participants were studied twice, 2–3 years apart. Resting energy expenditure was studied by indirect calorimetry, lean body mass and body fat by dual X-ray absorptiometry, and muscle mass was estimated based on 24-h urinary creatinine excretion. At baseline and 2–3 years later, body fat was significantly higher ( P<0.011 and P<0.003, respectively) and lean body mass significantly lower ( P<0.024 and P<0.012, respectively) in patients with Emery-Dreifuss muscular dystrophy as compared to subjects with hyperCKemia and healthy controls. Resting energy expenditure, over the study period, increased significantly in patients with Emery-Dreifuss muscular dystrophy ( P<0.031), but not in patients with hyperCKemia nor in healthy controls. Our study suggests that patients with Emery-Dreifuss muscular dystrophy may have increased energy expenditure relative to healthy subjects. If not met by increased caloric intake, this greater energy expenditure may partially contribute to a further deterioration in their muscle performance.