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. 2022 Jul;66(1):63-70.
doi: 10.1002/mus.27565. Epub 2022 May 27.

Body composition and myokines in a cohort of patients with Becker muscular dystrophy

Andrea Barp  1 , Elena Carraro  1 , Giovanni Goggi  2   3 , Andrea Lizio  1 , Alice Zanolini  1 , Carmelo Messina  4 , Silvia Perego  5 , Chiara Verdelli  6 , Giovanni Lombardi  5   7 , Valeria Ada Sansone #  1 , Sabrina Corbetta #  2   8
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Body composition and myokines in a cohort of patients with Becker muscular dystrophy

Andrea Barp et al. Muscle Nerve. 2022 Jul.

Abstract

Introduction/aims: Becker muscular dystrophy (BMD) is an X-linked disease leading to muscle wasting and weakness. The decrease in lean body mass (LBM) in Duchenne muscular dystrophy, has shown correlation with loss of muscle function and bone density (BD). Myokines (including irisin) are hormones secreted by skeletal muscle that allow crosstalk between muscle and bone. The present study analyzed body composition and circulating myokine levels in a cohort of BMD patients; moreover, the association between dual energy X-ray absorptiometry (DXA) parameters, functional motor assessments, and myokine levels was investigated.

Methods: All patients underwent DXA, blood samples for myokine assays, and functional motor assessments. A group of healthy controls (HCs) was also included.

Results: Thirty BMD patients, median age at evaluation 36.0 y [26.0-41.0], were included. Twenty-nine patients underwent whole-body DXA. Median value of total body Z-score was -0.70. The prevalence of low skeletal muscle mass defined as appendicular skeletal muscle mass index (ASMMI) < 7.59 kg/m2 was 83%. Irisin levels were significantly lower in BMD compared to HCs (p = .03). All DXA parameters showed significant correlation with the functional motor assessments, in particular the h2 -standardized lean mass lower limb index (p = .0006); h2 -standardized total fat mass showed negative correlations with North Star Ambulatory Assessment and 6 min walk test (p = .03).

Discussion: DXA is a useful tool to evaluate body composition in BMD patients; the decrease in BD and LBM is associated with a reduction of motor function in BMD.

Keywords: Becker muscular dystrophy; DXA; Duchenne muscular dystrophy; Irisin; bone mineral density.

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Conflict of interest statement

None of the authors has any conflict of interest to disclose.

Figures

FIGURE 1
FIGURE 1
Correlation between h2‐standardized lean mass lower limb index and functional motor assessments. The h2‐standardized lean mass lower limb index values showed significant positive correlation with 6MWT (A) and NSAA (B), and negative correlation with 10MWT (C), 10MRT (D), climb 4 steps (E), and rise from floor (Gowers) (F).
FIGURE 2
FIGURE 2
Correlation between bone mineral density of the lower limbs and irisin levels
See this image and copyright information in PMC

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References

    1. Hoffman EP, Kunkel LM, Angelini C, Clarke A, Johnson M, Harris JB. Improved diagnosis of Becker muscular dystrophy by dystrophin testing. Neurology. 1989;39(8):1011‐1017. doi:10.1212/wnl.39.8.1011 - DOI - PubMed
    1. Joyce NC, Hache LP, Clemens PR. Bone health and associated metabolic complications in neuromuscular diseases. Phys Med Rehabil Clin N Am. 2012;23(4):773‐799. doi:10.1016/j.pmr.2012.08.005 - DOI - PMC - PubMed
    1. Larson CM, Henderson RC. Bone mineral density and fractures in boys with Duchenne muscular dystrophy. J Pediatr Orthop. 2000;20(1):71‐74. - PubMed
    1. Palmieri GM, Bertorini TE, Griffin JW, Igarashi M, Karas JG. Assessment of whole body composition with dual energy x‐ray absorptiometry in Duchenne muscular dystrophy: correlation of lean body mass with muscle function. Muscle Nerve. 1996;19(6):777‐779. doi:10.1002/(SICI)1097-4598(199606)19:6<777::AID-MUS15>3.0.CO;2-I - DOI - PubMed
    1. Kanda F, Fujii Y, Takahashi K, Fujita T. Dual‐energy X‐ray absorptiometry in neuromuscular diseases. Muscle Nerve. 1994;17(4):431‐435. doi:10.1002/mus.880170411 - DOI - PubMed

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