Abstract
PurposeThe purpose of this study was: 1) To compare Resting energy expenditure (REE) in adult males with Becker’s Muscular Dystrophy (BeMD, n = 21, 39 ±12 years) and healthy controls (CTRL, n = 12, 37 ±12 years) 2) Determine whether other physiological parameters correlate with REE in BeMD, and 3) Compare current prediction methods of REE with measured REE.MethodsREE was calculated via indirect calorimetry using continuous, expired gas analysis following an overnight fast. Fat free mass (FFM) and fat mass were measured by bioelectrical impedance. B-mode ultrasound measured Tibialis Anterior (TA) and Gastrocnemius Medialis (GM) anatomical cross sectional area (ACSA). The Bone Specific Physical Activity Questionnaire measured physical activity.ResultsNo difference in REE was found between CTRL and BeMD groups (1913 ±203 & 1786 ±324 Kcal respectively). Other physiological comparisons showed increased fat mass (+54%), decreased TA ACSA (-42%), increased GM ACSA (+25%) as well as reduced respiratory function (FVC -28%; FEV1−27%) in BeMD adults compared to controls. REE estimated from prediction equations (Schofield’s) in Muscular Dystrophy were different from measured REE (P<0.05, bias = -728kcal), while the Mifflin equation was no different from measured REE (r2 = 0.58, Bias = -8kcal). Within the present BeMD, REE predicted from FFM (REE = FFM x 34.57–270; r2 = 0.85) and body mass (REE = BM x 15.65 + 421.5; r2 = 0.66), were not different from measured REE (bias equals 0 and 0.2kcals, respectively)ConclusionsDespite no differences in REE between CTRL and BeMD adults, increased fat masses highlights the requirement for explicit nutritional guidelines, as well as maintenance of physical activity levels, where possible. Prediction equations are frequently used in clinical settings, however these have been shown to be less accurate in BeMD; therefore, the equations proposed here should be used where possible.
Highlights
Muscular dystrophy (MD) is a broad group of myogenic recessive muscle disorders, with variable severity [1]
Despite no differences in Resting energy expenditure (REE) between CTRL and Becker’s muscular Dystrophy (BeMD) adults, increased fat masses highlights the requirement for explicit nutritional guidelines, as well as maintenance of physical activity levels, where possible
Fat% and fat mass were 43% and 54% greater in BeMD participants compared to CTRL, respectively (P
Summary
Muscular dystrophy (MD) is a broad group of myogenic recessive muscle disorders, with variable severity [1]. Duchenne (DMD) and Beckers (BeMD) Muscular Dystrophy are characterised by the absence or reduced expression of the cytoskeletal protein dystrophin, resulting in progressive muscle degeneration[2]. Duchenne Muscular Dystrophy (DMD), characterised by non-functioning dystrophin, is the most severe form of MD, with an estimated incidence of 3 in 100,000 boys [3]. Becker’s muscular Dystrophy (BeMD) evidences partially functioning dystrophin, and is a milder yet more variable form of dystrophinopathy, with an incidence of 2 in 100,000 male births [3]. Shimizu-Fujiwara et al [4] estimated that children with DMD had decreasing physical activity levels, resulting in reduced calorific energy requirements, reducing their daily calorific intake needs. The variable nature and relative degree of physical disability within the BeMD population may be less suitable to a “one size fits all” approach to daily calorific guidelines
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