Assessment of whole body composition with dual energy X-ray absorptiometry in Duchenne muscular dystrophy: Correlation of lean body mass with muscle function

Palmieri, Genaro M. A.; Bertorini, Tulio E.; Griffin, Judy W; Igarashi, Masanori; Karas, James G.

doi:10.1002/(sici)1097-4598(199606)19:6<777::aid-mus15>3.0.co;2-i

Cited by 60 publications

(42 citation statements)

References 13 publications

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“…In accordance with previous studies, our protocol confirmed a great alteration of body composition in DMD boys (LeroyWillig et al, 1997;Palmieri et al, 1996). Mean fat mass was 32%, a value which is similar to data obtained using dual X-ray absorptiometry (31.7 AE 17.9) by Palmieri et al (1996) and using MRI (37.7 AE 6.6) by Leroy-Willig et al (1997) in DMD children of the same age (Table 3).…”

Section: Discussionsupporting

confidence: 91%

“…Mean fat mass was 32%, a value which is similar to data obtained using dual X-ray absorptiometry (31.7 AE 17.9) by Palmieri et al (1996) and using MRI (37.7 AE 6.6) by Leroy-Willig et al (1997) in DMD children of the same age (Table 3). Dividing our subjects into two subgroups (normal weight and obese patients), we observed that only obese children showed a markedly increased fat mass (more than 50%) while NODMD showed fat mass values similar to normal ones (Table 2).…”

Section: Discussionsupporting

confidence: 80%

“…Unpaired Student's t-test was used to compare mean differences in body composition and energy expenditure between obese and non-obese Duchenne children and between patients and healthy children of the same age. The values of body composition and energy expenditure of healthy children were obtained from the literature (Leroy- Willig et al, 1997;Palmieri et al, 1996;Hankard, 1998, Maffeis et al, 1991Weinsier et al, 1992). Significance was established at P < 0.05.…”

Section: Discussionmentioning

confidence: 99%

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Body composition and energy expenditure in Duchenne muscular dystrophy

et al. 2003

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Objective: To investigate the relationship between resting energy expenditure (REE) and body composition in Duchenne Muscular Dystrophy (DMD). Design: An observational study. Setting: University Research Centre. Subjects: Nine Duchenne children (age range 6 -12 y), mean relative weight 128%, agreed to undergo the investigation and all of them completed the study; Interventions: Assessment of body composition (total body fat and skeletal muscle mass) by magnetic resonance imaging and resting energy expenditure by indirect calorimetry. Main outcome measures: Fat mass (FM; kg and percentage weight), fat-free mass (FFM; kg and percentage weight), muscle mass (kg and percentage weight), resting energy expenditure (kJ=kg body weight and kJ=kg fat-free mass). Results: In Duchenne children fat mass averages 32% and total skeletal muscle mass 20% of body weight. Resting energy expenditure per kg of body weight falls within the normal range for children of the same age range, while when expressed per kg of FFM is significantly higher than reference values. No relationship was found between REE and total skeletal muscle mass. Conclusions:Our results do not demonstrate a low REE in DMD boys; on the contrary REE per kg of FFM is higher than normal, probably due to the altered FFM composition. We suggest that the development of obesity in DMD children is not primarily due to a low REE but to other causes such as a reduction in physical activity and or overfeeding.

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Section: Discussionsupporting

confidence: 91%

Section: Discussionsupporting

confidence: 80%

Section: Discussionmentioning

confidence: 99%

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Body composition and energy expenditure in Duchenne muscular dystrophy

et al. 2003

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“…Increases in muscle strength and activity induced by prednisone treatment may stabilize bone density, as supported by the current study and postulated previously. 32,[37][38][39][40] However, a longer study would be required to adequately assess the effects of corticosteroid use on bone metabolism and fracture risk. Furthermore, we did not assess femoral bone density, which has been shown to be abnormal even in younger ambulant boys with DMD and to correlate with increased risk of lower extremity fractures.…”

Section: Discussionmentioning

confidence: 99%

Randomized, blinded trial of weekend vs daily prednisone in Duchenne muscular dystrophy

et al. 2011

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Objective: To perform a double-blind, randomized study comparing efficacy and safety of daily and weekend prednisone in boys with Duchenne muscular dystrophy (DMD). Methods:A total of 64 boys with DMD who were between 4 and 10 years of age were randomized at 1 of 12 centers of the Cooperative International Neuromuscular Research Group. Efficacy and safety of 2 prednisone schedules (daily 0.75 mg/kg/day and weekend 10 mg/kg/wk) were evaluated over 12 months.Results: Equivalence was met for weekend and daily dosing of prednisone for the primary outcomes of quantitative muscle testing (QMT) arm score and QMT leg score. Secondary strength scores for QMT elbow flexors also showed equivalence between the 2 treatment groups. Overall side effect profiles of height and weight, bone density, cataract formation, blood pressure, and behavior, analyzed at 12 months, did not differ between weekend and daily dosing of prednisone. Conclusions:Weekend dosing of prednisone is equally beneficial to the standard daily dosing of prednisone. Analysis of side effect profiles demonstrated overall tolerability of both dosing regimens. Classification of evidence:This study provides Class I evidence that weekend prednisone dosing is as safe and effective as daily prednisone in preserving muscle strength and preventing body mass index increases in boys with DMD over a 12-month period. Neurology Duchenne muscular dystrophy (DMD) is a progressive muscle disorder due to mutations in the dystrophin gene.1,2 Current treatments can slow disease progression, prolonging ambulation, and improving quality of life and survival. [3][4][5] Corticosteroid treatment for DMD 6-12 is recommended by an American Academy of Neurology practice parameter.13 Furthermore, a recently published standard of care review emphasized the benefit of corticosteroids for DMD.14,15 In a large DMD natural history study currently run by the Cooperative International Neuromuscular Research Group (CINRG), 85% of participants are steroid-treated. 16,17 We hypothesized that weekend prednisone dosing would provide equally effective treatment for DMD as standard daily dosing. Furthermore, corticosteroids might be more widely used in DMD if a dosing regimen had fewer side effects, including less weight gain, less effect on linear Editorial, page 416Podcast Scan this code with your smartphone to access this feature CME Scan this code with your smartphone to access this feature

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“…Dietary intake and undernutrition in preschool children with cerebral palsy: comparison between high-and low-resource countries K BENFER 1,2 Participants and Setting: Hundred and eighty children with CP aged 18 to 36 months, 81 children born in Bangladesh (mean age=27.6mo, 50 males, GMFCS I-II=23.5%, III=30.9%, IV-V=45.7%) and 99 children born in Queensland, Australia (mean age=27.1mo, 65 males, GMFCS I-II=56.2%, III=17.7%, IV-V=26.2%). Methods: Parents reported on their child's dietary intake using a 1 day (Bangladesh, BD) and 3 day (Australia, AU) weighed record.…”

Section: Sp7mentioning

confidence: 78%

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Assessment of whole body composition with dual energy X-ray absorptiometry in Duchenne muscular dystrophy: Correlation of lean body mass with muscle function

Cited by 60 publications

References 13 publications

Body composition and energy expenditure in Duchenne muscular dystrophy

Body composition and energy expenditure in Duchenne muscular dystrophy

Randomized, blinded trial of weekend vs daily prednisone in Duchenne muscular dystrophy

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