title: Increased resting energy expenditure in subjects with Emery-Dreifuss muscular dystrophy.
abstract: 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.
authors.affiliation: Unit of Clinical Nutrition, Tel-Aviv Sourasky Medical Centre, 6 Weizmann Street, Tel-Aviv 64239, Israel. vaisman@tasmc.health.gov.il
authors.forename: N
authors.initials: N
authors.lastname: Vaisman
authors.forename: S
authors.initials: S
authors.lastname: Katzenellenbogen
authors.forename: Y
authors.initials: Y
authors.lastname: Nevo
mesh_terms: D015502:Absorptiometry, Photon; D000273:Adipose Tissue / Q000378:metabolism / Q000473:pathology / Q000503:physiopathology; D000328:Adult; D001284:Atrophy / Q000378:metabolism / Q000473:pathology / Q000503:physiopathology; D015992:Body Mass Index; D003404:Creatinine / Q000652:urine; D004734:Energy Metabolism / Q000235:genetics*; D006801:Humans; D006934:Hypercalcemia / Q000378:metabolism / Q000503:physiopathology; D006947:Hyperkalemia / Q000378:metabolism / Q000503:physiopathology; D008137:Longitudinal Studies; D008297:Male; D018482:Muscle, Skeletal / Q000378:metabolism* / Q000473:pathology / Q000503:physiopathology; D020389:Muscular Dystrophy, Emery-Dreifuss / Q000378:metabolism* / Q000473:pathology / Q000503:physiopathology; D012016:Reference Values; D012146:Rest / Q000502:physiology; D015854:Up-Regulation / Q000235:genetics*
chemical_list: D003404:Creatinine