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Measurements of Motor Function and Other Clinical Outcome Parameters in Ambulant Children with Duchenne Muscular Dystrophy
Published on: January 12, 2019
Bone density and bone health alteration in boys with Duchenne Muscular Dystrophy: a prospective observational study
Renu Suthar1, B V Chaithanya Reddy1, Manisha Malviya1
1Pediatric Neurology Unit, Department of Pediatrics, APC, PGIMER, Chandigarh, India.
Insights
Bone health is significantly compromised in boys with Duchenne Muscular Dystrophy (DMD), with over half experiencing low bone mineral density and nearly all showing vitamin D deficiency. Longer glucocorticoid use is linked to reduced bone density.
Area of Science:
- Pediatric Endocrinology
- Bone Metabolism
- Neuromuscular Disorders
Background:
- Boys with Duchenne Muscular Dystrophy (DMD) face heightened risks of skeletal complications, including fractures.
- Glucocorticoid therapy, common in DMD management, can negatively impact bone health.
Purpose of the Study:
- To evaluate bone health parameters in North Indian boys diagnosed with DMD.
- To identify risk factors associated with compromised bone health in this population.
Main Methods:
- A prospective observational study involving 76 ambulatory boys with DMD on glucocorticoid therapy.
- Bone health assessment included X-ray, DXA scans, and biochemical markers (serum calcium, vitamin D metabolites, osteocalcin, osteopontin, Ntx).
Main Results:
- Over 70% of boys had low bone mineral density (BMD) at the lumbar spine and femoral neck.
- Vitamin D deficiency (25[OH]D) was prevalent in 89.5% of participants, with universal deficiency in 1,25(OH)2D3.
- Longer duration of glucocorticoid therapy correlated with lower BMD (p=0.04).
Conclusions:
- Bone health is significantly impaired in North Indian boys with DMD.
- Reduced BMD and widespread vitamin D deficiency are critical concerns.
- Prolonged glucocorticoid treatment emerges as a significant risk factor for low BMD in DMD patients.
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.
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