However, there is currently no research available in either muscle mass or REE in people with BeMD of any age. Loss of muscle mass is a consequence of the progression of MDs, however lower physical activity levels associated with forms of MD may also lead to increased muscle atrophy. It is likely therefore, that REE will be lower in adults with BeMD, who also experience a loss of muscle mass. In DMD, the loss of FFM is attributed with a lower REE than non-dystrophic controls, with the progressive nature of DMD, resulting in the loss of FFM, leads to REE to decrease with age within DMD. In addition, REE can be normalised to FFM to reduce the impact of body size on REE. REE, here defined as the energy used in maintaining bodily functions such as respiration, circulation, cellular metabolism and body temperature in a fasted, thermo-neutral state, is best predicted in healthy population by FFM, with correlations ranging between 65–90%.
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However, unlike DMD, there are no current studies of REE or its estimation from anthropometric measures such as body mass or fat free mass in adult males with BeMD. Nutritional support and knowledge of Resting energy expenditure (REE) is essential for individuals with limited physical activity in order to provide guidance on calorific intake and to limit excessive weight gain. In contrast, 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. Shimizu-Fujiwara et al estimated that children with DMD had decreasing physical activity levels, resulting in reduced calorific energy requirements, therefore reducing their daily calorific intake needs. Becker’s muscular Dystrophy (BeMD) evidences partially functioning dystrophin, and is therefore a milder yet more variable form of dystrophinopathy, with an incidence of 2 in 100,000 male births. 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. 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. Muscular dystrophy (MD) is a broad group of myogenic recessive muscle disorders, with variable severity. This is an open access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.ĭata Availability: All relevant data are within the paper and its Supporting Information files.įunding: The author(s) received no specific funding for this work.Ĭompeting interests: The authors have declared that no competing interests exist. Received: OctoAccepted: DecemPublished: January 6, 2017Ĭopyright: © 2017 Jacques et al.
PLoS ONE 12(1):Įditor: Diego Fraidenraich, Rutgers University Newark, UNITED STATES
Citation: Jacques MF, Orme P, Smith J, Morse CI (2017) Resting Energy Expenditure in Adults with Becker’s Muscular Dystrophy.