Abstract
Objectives
Boys with Duchenne Muscular Dystrophy (DMD) are at increased risk for compromised bone health, manifesting as low-impact trauma long bone fractures and vertebral compression fractures.
Methods
In a prospective observational study, we studied bone health parameters in North Indian boys with DMD. We consecutively enrolled ambulatory boys with DMD on glucocorticoid therapy. Bone health was evaluated with X-ray spine, Dual-energy X-ray absorptiometry (DXA), serum calcium, vitamin D3 (25[OH]D), 1,25-dihyroxyvitamin D3 (1,25[OH]2D3), serum osteocalcin, osteopontin, and N terminal telopeptide of type 1 collagen (Ntx) levels.
Results
A total of 76 boys with DMD were enrolled. The median age was 8.5 (interquartile range [IQR] 7.04–10.77) years. Among these, seven (9.2%) boys had long bone fractures, and four (5.3%) had vertebral compression fractures. Fifty-four (71%) boys underwent DXA scan, and among these 31 (57%) had low bone mineral density (BMD, ≤−2 z-score) at the lumbar spine. The mean BMD z-score at the lumbar spine was −2.3 (95% confidence interval [CI] = −1.8, −2.8), and at the femoral neck was −2.5 (95% CI = −2, −2.9). 25(OH)D levels were deficient in 68 (89.5%, n=76) boys, and 1,25(OH)2D3 levels were deficient in all. Mean serum osteocalcin levels were 0.68 ± 0.38 ng/mL (n=54), serum osteopontin levels were 8.6 ± 4.6 pg/mL (n=54) and serum Ntx levels were 891 ± 476 nmol/L (n=54). Boys with low BMD received glucocorticoids for longer duration, in comparison to those with normal BMD (median, IQR [16.9 (6–34) months vs. 7.8 (4.8–13.4) months]; p=0.04).
Conclusions
Bone health is compromised in North Indian boys with DMD. BMD at the lumbar spine is reduced in more than half of boys with DMD and nearly all had vitamin D deficiency on regular vitamin D supplements. Longer duration of glucocorticoid therapy is a risk factor for low BMD in our cohort.
Funding source: PGI/Intramural Research
Award Identifier / Grant number: 71/2-Edu-16/236
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Research funding: PGI/Intramural Research grant/no. 71/2-Edu-16/236.
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Author contributions: All the authors have accepted responsibility for the entire content of this submitted manuscript and approved submission.
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Competing interests:
No funding organizations played a role in the study design; in the collection, analysis, and interpretation of data; in the writing of the report; or in the decision to submit the report for publication.
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Informed consent: Informed consent from the parents and assent from the child was obtained.
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Ethical approval: The study was approved from the institute ethics committee with reference no. NK/4402/Study/282 (dated 05/01/2018).
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Availability of data and material: Data will be deposited for review purpose.
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Code availability: Codes will be provided with the data.
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Supplementary Material
The online version of this article offers supplementary material (https://doi.org/10.1515/jpem-2020-0680).
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