Body composition of patients with Duchenne muscular dystrophy: the Greek experience

Doulgeraki, Artemis; Athanasopoulou, Helen; Katsalouli, Marina; Petrocheilou, Glykeria; Paspati, Ioanna; Monopolis, Ioannis

doi:10.1007/s13760-015-0582-1

Cited by 9 publications

(9 citation statements)

References 27 publications

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“…Body composition parameters are similarly difficult to compare to a growing, healthy, non-glucocorticoid-using population, with Z-scores again being inappropriate to describe our cohort. We did however see a mild increase in overall lean body mass and no significant increase in FMI Z-score or mean percentage change in FMI during induction of puberty, compared with another report [23]. This may suggest a positive anabolic effect of testosterone on body mass indices.…”

Section: Discussion/conclusioncontrasting

confidence: 81%

Effect of Testosterone Treatment for Delayed Puberty in Duchenne Muscular Dystrophy

et al. 2020

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Objective: To evaluate the impact of pubertal induction with testosterone on bone health, body composition, and motor function in boys with Duchenne muscular dystrophy (DMD) receiving long-term glucocorticoid. Study Design: A retrospective, observational, pre-post study investigating the impact of testosterone therapy on bone mass accrual, vertebral fracture incidence, body composition, motor function, and quality of life in boys with DMD. All those boys aged ≥14 years, on chronic steroid therapy, who had delayed puberty, and were receiving oral testosterone or oral and then transitioned to intramuscular testosterone, to complete virilization, were included. Prior/concomitant zoledronic acid use was included. The primary outcome was lumbar spine areal bone mineral density (BMD LS). Results: Puberty was induced, using oral testosterone undecanoate in 16 individuals, 10 of whom had transited to intramuscular testosterone at time of assessment. Median age at testosterone onset was 14.5 years (range 14-17.7). Median duration of testosterone therapy was 2.5 years (range 1.0-4.5). There was statistically significant increase in median BMD LS (0.523-0.700, p < 0.001) and median annualized percentage change of BMD LS (-1.34 to +10.08%, p < 0.001), with median Tanner stage 4 at evaluation (range 2-4). Ten of 14 assessed had no progression in vertebral fractures. Fat mass index (FMI) standard deviation score (SDS), lean body mass index (LBMI) SDS, and percentage change of FMI and LBMI were statistically unchanged. Cardiac function remained stable. Motor function in non-ambulatory individuals with Egen Klassifikation scores improved in 7 of 8. Conclusion: Testosterone for delayed puberty acted as an adjunct to bisphosphonates to increase bone density and stabilize vertebral fracture in most boys with DMD.

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Section: Discussion/conclusioncontrasting

confidence: 81%

Effect of Testosterone Treatment for Delayed Puberty in Duchenne Muscular Dystrophy

et al. 2020

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“…Inclusion of a whole-body scan into this annual examination would present minimal additional burden for the patient. Furthermore, several studies have demonstrated a relationship between DXA-derived lean body mass measures and muscle strength in patients with DMD, in addition to demonstrating that males with DMD have lower lean body mass as compared with heathy males 13 – 15 , 27 – 29 .…”

Section: Discussionmentioning

confidence: 99%

Dual-energy X-ray absorptiometry measures of lean body mass as a biomarker for progression in boys with Duchenne muscular dystrophy

Sherlock

Palmer

Wagner

et al. 2022

Sci Rep

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We evaluated whether whole-body dual-energy X-ray absorptiometry (DXA) measures of lean body mass can be used as biomarkers for disease progression and treatment effects in patients with Duchenne muscular dystrophy. This post hoc analysis utilized data from a randomized, 2-period study of domagrozumab versus placebo in 120 ambulatory boys with DMD. DXA measures of lean body mass were obtained from the whole body (excluding head), arms, legs and appendicular skeleton at baseline and every 16 weeks. Treatment effects on DXA measures for domagrozumab versus placebo were assessed at Week 49. At Week 49, domagrozumab statistically significantly increased lean body mass versus placebo in the appendicular skeleton (p = 0.050) and arms (p < 0.001). The relationship between lean body mass at Week 49 and functional endpoints at Week 97 was evaluated. Changes in lean body mass at Week 49 in all regions except arms were significantly correlated with percent change from baseline in 4-stair climb (4SC) at Week 97. DXA-derived percent lean mass at Week 49 also correlated with 4SC and North Star Ambulatory Assessment at Week 97. These data indicate that whole-body DXA measures can be used as biomarkers for treatment effects and disease progression in patients with DMD, and warrant further investigation.Trial registration: ClinicalTrials.gov, NCT02310763; registered 8 December 2014.

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“…Both ALM and whole body lean mass (WBLM) have been shown to be associated with muscle strength in individuals with DMD 3,4 . Cross‐sectional studies have reported a lower percentage of ALM and WBLM in males with DMD compared to healthy males, 3,5‐7 but these studies had small sample sizes or did not investigate age‐related changes in lean mass.…”

Section: Introductionmentioning

confidence: 99%

Age‐related changes in appendicular lean mass in males with Duchenne muscular dystrophy: A retrospective review

et al. 2020

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Background Appendicular lean mass (ALM) trajectory in males with Duchenne muscular dystrophy (DMD) has potential applicability for treatment and research and has not been characterized. Methods This chart review included longitudinal data on 499 males with DMD receiving glucocorticoids and 693 controls, ages 5 to 22.9 y. ALM (kg) was measured by dual energy x‐ray absorptiometry (DXA). Appendicular lean mass index (ALMI, kg/m2) was calculated for height adjustment. Reference centiles were generated using data from healthy controls, and ALM and ALMI Z‐scores were calculated for patients with DMD. Generalized linear models were used to estimate median Z‐scores by age and functional mobility status (FMS) score. ALM velocity by age was modeled using superimposition, translation and rotation (SITAR). Results Compared to controls, males with DMD had lower ALM from an early age. ALMI Z‐scores dropped below 0 at age 8 y or FMS of 2, and below −2.0 at age 13 y or FMS of 3 (P < .05). Age at peak ALM velocity was similar in both groups, but the magnitude was higher in controls (3.5 vs. 0.7 kg/y, P < .0001). Patients with DMD had a transient loss of ALM around age 12 y, an increase at age 14 y, then a further decline at age 16 y, remaining low thereafter. Conclusions Males with DMD have progressive decline in lean mass with age and worsening functional mobility. DXA measurement of ALM may be useful for monitoring lean mass status in patients with DMD, providing valuable information for individual treatment plans and research endeavors.

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Body composition of patients with Duchenne muscular dystrophy: the Greek experience

Cited by 9 publications

References 27 publications

Effect of Testosterone Treatment for Delayed Puberty in Duchenne Muscular Dystrophy

Effect of Testosterone Treatment for Delayed Puberty in Duchenne Muscular Dystrophy

Dual-energy X-ray absorptiometry measures of lean body mass as a biomarker for progression in boys with Duchenne muscular dystrophy

Age‐related changes in appendicular lean mass in males with Duchenne muscular dystrophy: A retrospective review

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