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. 2024 Aug;70(2):226-231.
doi: 10.1002/mus.28173. Epub 2024 Jun 5.

Appendicular lean mass index and motor function in ambulatory patients with Duchenne muscular dystrophy

Michael Kiefer  1   2   3 , Elise Townsend  1 , Celina Goncalves  2 , K Courtney Shellenbarger  2 , Perman Gochyyev  1 , Brenda L Wong  2
Affiliations

Appendicular lean mass index and motor function in ambulatory patients with Duchenne muscular dystrophy

Michael Kiefer et al. Muscle Nerve. 2024 Aug.

Abstract

Introduction/aims: Appendicular lean mass index (ALMI) has been linked to motor function in patients with Duchenne muscular dystrophy (DMD). However, quantification of the relationship between ALMI and disease-specific clinical outcome assessment trajectories is needed. The purpose of this study was to determine associations between dual-energy x-ray absorptiometry (DXA) derived estimates of ALMI and motor function in ambulatory patients with DMD.

Methods: A retrospective analysis of longitudinal clinical visit data from 137 glucocorticoid-treated patients with DMD collected via structured motor assessment protocol evaluated associations between ALMI and motor function indexed by the North Star Ambulatory Assessment (NSAA) and 10 Meter Walk/run Test (10MWT). Body composition was assessed using DXA. ALMI was calculated by dividing arm and leg lean mass by height in m2; fat mass index (FMI) was calculated by dividing whole body fat mass by height in m2. Linear mixed-effects models were used to estimate associations between ALMI and motor function, controlling for age and FMI.

Results: The full prediction model (age, age,2 ALMI, and FMI) explained 57% of the variance in NSAA scores and 63% of the variance in 10MWT speed. A 1 kg/m2 higher ALMI value predicted a 5.4-point higher NSAA score (p < .001) and 0.45 m/s faster 10MWT speed (p < .001). A 1 kg/m2 higher FMI value predicted a 1.5-point lower NSAA score (p < .001) and 0.14 meters/second slower 10MWT speed (p < .001).

Discussion: DXA-derived estimates of ALMI and FMI are associated with motor function in DMD and may explain variation in DMD disease progression.

Keywords: Duchenne muscular dystrophy; appendicular lean mass index; body composition; dual‐energy x‐ray absorptiometry; fat mass index.

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References

REFERENCES

    1. Birnkrant DJ, Bushby K, Bann CM, et al. Diagnosis and management of Duchenne muscular dystrophy, part 1: diagnosis, and neuromuscular, rehabilitation, endocrine, and gastrointestinal and nutritional management. Lancet Neurol. 2018;17(3):251‐267. doi:10.1016/S1474‐4422(18)30024‐3
    1. Duan D, Goemans N, Takeda S, Mercuri E, Aartsma‐Rus A. Duchenne muscular dystrophy. Nat Rev Dis Primers. 2021;7(1):13. doi:10.1038/s41572‐021‐00248‐3
    1. Matthews E, Brassington R, Kuntzer T, Jichi F, Manzur AY. Corticosteroids for the treatment of Duchenne muscular dystrophy. Cochrane Database Syst Rev. 2016;2016(5):CD003725. doi:10.1002/14651858.CD003725.pub4
    1. Goemans N, vanden Hauwe M, Signorovitch J, Swallow E, Song J, Collaborative Trajectory Analysis Project (cTAP). Individualized prediction of changes in 6‐minute walk distance for patients with Duchenne muscular dystrophy. PLoS One. 2016;11(10):e0164684. doi:10.1371/journal.pone.0164684
    1. Billich N, Adams J, Carroll K, et al. The relationship between obesity and clinical outcomes in young people with Duchenne muscular dystrophy. Nutrients. 2022;14(16):3304. doi:10.3390/nu14163304

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