Low bone density and bone metabolism alterations in Duchenne muscular dystrophy: response to calcium and vitamin D treatment

2010 ◽  
Vol 22 (2) ◽  
pp. 529-539 ◽  
Author(s):  
M. L. Bianchi ◽  
L. Morandi ◽  
E. Andreucci ◽  
S. Vai ◽  
J. Frasunkiewicz ◽  
...  
2011 ◽  
Vol 26 (8) ◽  
pp. 1891-1903 ◽  
Author(s):  
Anna Rufo ◽  
Andrea Del Fattore ◽  
Mattia Capulli ◽  
Francesco Carvello ◽  
Loredana De Pasquale ◽  
...  

Bone ◽  
2009 ◽  
Vol 45 ◽  
pp. S72
Author(s):  
S. Vai ◽  
L. Morandi ◽  
M.L. Bianchi

Bone ◽  
2010 ◽  
Vol 46 ◽  
pp. S79-S80
Author(s):  
Anna Rufo ◽  
Andrea Del Fattore ◽  
Maria Bianchi ◽  
Enrico Bertini ◽  
Antonio Musarò ◽  
...  

Bone ◽  
2009 ◽  
Vol 44 ◽  
pp. S237-S238 ◽  
Author(s):  
A. Rufo ◽  
A. Del Fattore ◽  
M.L. Bianchi ◽  
L. Morandi ◽  
E. Bertini ◽  
...  

2016 ◽  
Vol 101 (10) ◽  
pp. 957-961 ◽  
Author(s):  
Nahla Alshaikh ◽  
Andreas Brunklaus ◽  
Tracey Davis ◽  
Stephanie A Robb ◽  
Ros Quinlivan ◽  
...  

AimAssessment of the efficacy of vitamin D replenishment and maintenance doses required to attain optimal levels in boys with Duchenne muscular dystrophy (DMD).Method25(OH)-vitamin D levels and concurrent vitamin D dosage were collected from retrospective case-note review of boys with DMD at the Dubowitz Neuromuscular Centre. Vitamin D levels were stratified as deficient at <25 nmol/L, insufficient at 25–49 nmol/L, adequate at 50–75 nmol/L and optimal at >75 nmol/L.Result617 vitamin D samples were available from 197 boys (range 2–18 years)—69% from individuals on corticosteroids. Vitamin D-naïve boys (154 samples) showed deficiency in 28%, insufficiency in 42%, adequate levels in 24% and optimal levels in 6%. The vitamin D-supplemented group (463 samples) was tested while on different maintenance/replenishment doses. Three-month replenishment of daily 3000 IU (23 samples) or 6000 IU (37 samples) achieved optimal levels in 52% and 84%, respectively. 182 samples taken on 400 IU revealed deficiency in 19 (10%), insufficiency in 84 (47%), adequate levels in 67 (37%) and optimal levels in 11 (6%). 97 samples taken on 800 IU showed deficiency in 2 (2%), insufficiency in 17 (17%), adequate levels in 56 (58%) and optimal levels in 22 (23%). 81 samples were on 1000 IU and 14 samples on 1500 IU, with optimal levels in 35 (43%) and 9 (64%), respectively. No toxic level was seen (highest level 230 nmol/L).ConclusionsThe prevalence of vitamin D deficiency and insufficiency in DMD is high. A 2-month replenishment regimen of 6000 IU and maintenance regimen of 1000–1500 IU/day was associated with optimal vitamin D levels. These data have important implications for optimising vitamin D dosing in DMD.


2021 ◽  
Vol 2 (1) ◽  
pp. 38-50
Author(s):  
Tatiana A. Gremiakova ◽  
Vasiliy M. Souslov ◽  
Gulzhan E. Sakbaeva ◽  
Andrey A. Stepanov

Duchenne muscular dystrophy (DMD) is an X-linked recessive degenerative neuromuscular disorder due to a deficiency of dystrophin protein. This protein is most common in skeletal and cardiac muscles, to a lesser extent in smooth muscles and the brain. With DMD, progressive damage and muscle degeneration, a delay in motor development, and respiratory cardiac disorders are progressing. Patients with DMD have an increased risk of developing osteoporosis, fractures of the tubular bones and vertebrae, and neurocognitive impairment. Vitamin D is recommended prophylactically for DMD since many studies have shown its deficiency. The purpose of this work is to consolidate the literature data on the vitamin D deficiency in DMD patients and its effects on the development of concurrent comorbid conditions of the musculoskeletal, endocrine, and nervous systems. The authors discuss data concerning the appropriate level of vitamin D throughout the life span of DMD has a positive effect on the course of the disease patients’ quality of life ends. Primary clinical outcomes of vitamin D normalization include prevention of the development of osteoporosis (especially after the start of steroid therapy), fractures of the tubular bones and vertebrae, prolonged ability to walk, more effective treatment with bisphosphonates, including a decrease in the number of complications during initial use and lower jaw necrosis, positive effect on the expression of autistic spectrum symptoms. For patients with long-term steroid therapy, metabolic and liver disorders, calcidiol could be used, allowing quick deficiency compensation instead of standard vitamin D preparations.


2020 ◽  
Vol 26 (12) ◽  
pp. 1477-1485
Author(s):  
Wen-bin Zheng ◽  
Yi Dai ◽  
Jing Hu ◽  
Di-chen Zhao ◽  
Ou Wang ◽  
...  

Objective: Duchenne muscular dystrophy (DMD) is a severe X-linked progressive neuromuscular disease that brings a significantly increased risk of osteoporosis and bone fractures. We prospectively evaluated the effects of oral and intravenous bisphosphonates on the bones of children with DMD. Methods: This study included a total of 52 children with DMD. They were divided into zoledronic acid (ZOL), alendronate (ALN), and control groups according to bone mineral density (BMD) and history of fragility fractures. For 2 years, all patients took calcium, vitamin D, and calcitriol. Meanwhile, 17 patients received infusions of ZOL, and 18 patients received ALN. BMD, serum levels of alkaline phosphatase (ALP) and the cross-linked C-telopeptide of type I collagen (β-CTX) were evaluated. Results: After 24 months of treatment, the percentage changes in lumbar spine BMD were 23.2 ± 9.7% and 23.6 ± 8.8% in the ZOL and ALN groups (all P<.01 vs. baseline). The increases did not differ between the ZOL and ALN groups, but were significantly larger than those of the control group ( P<.01). Serum β-CTX and ALP levels, respectively, were decreased by 44.4 ± 18.0% and 31.9 ± 26.7% in the ZOL group and by 36.0 ± 20.3% and 25.8 ± 14.4% in the ALN group (all P<.01 vs. baseline). Conclusion: Zoledronic acid and alendronate had similar protective effects to increase bone mineral density and reduce bone resorption in children with DMD, which were superior to treatment of calcium, vitamin D, and calcitriol. Abbreviations: 25OHD = 25 hydroxyvitamin D; ALN = alendro-nate; ALP = alkaline phosphatase; ALT = alanine aminotransferase; BMD = bone mineral density; BP = bisphosphonate; Ca = calcium; β-CTX = cross-linked C-telopeptide of type I collagen; DMD = Duchenne muscular dystrophy; FN = femoral neck; GC = glucocorticoid; LS = lumbar spine; ZOL = zoledronic acid


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