scholarly journals Optimizing Bone Health in Duchenne Muscular Dystrophy

2015 ◽  
Vol 2015 ◽  
pp. 1-9 ◽  
Author(s):  
Jason L. Buckner ◽  
Sasigarn A. Bowden ◽  
John D. Mahan
Keyword(s):  
Duchenne Muscular Dystrophy ◽  
Muscular Dystrophy ◽  
Bone Mass ◽  
Bone Health ◽  
Fragility Fractures ◽  
Premature Death ◽  
Screening Methods ◽  
Mineral Density ◽  
Progressive Muscle Weakness ◽  
Spinal Imaging

Duchenne muscular dystrophy (DMD) is an X-linked recessive disorder characterized by progressive muscle weakness, with eventual loss of ambulation and premature death. The approved therapy with corticosteroids improves muscle strength, prolongs ambulation, and maintains pulmonary function. However, the osteoporotic impact of chronic corticosteroid use further impairs the underlying reduced bone mass seen in DMD, leading to increased fragility fractures of long bones and vertebrae. These serious sequelae adversely affect quality of life and can impact survival. The current clinical issues relating to bone health and bone health screening methods in DMD are presented in this review. Diagnostic studies, including biochemical markers of bone turnover and bone mineral density by dual energy X-ray absorptiometry (DXA), as well as spinal imaging using densitometric lateral spinal imaging, and treatment to optimize bone health in patients with DMD are discussed. Treatment with bisphosphonates offers a method to increase bone mass in these children; oral and intravenous bisphosphonates have been used successfully although treatment is typically reserved for children with fractures and/or bone pain with low bone mass by DXA.

scholarly journals Effects of Bisphosphonate Therapy on Bone Mineral Density in Boys with Duchenne Muscular Dystrophy

2020 ◽  
Vol 13 ◽  
pp. 117955142097240
Author(s):  
Rebecca Ronsley ◽  
Nazrul Islam ◽  
Mehima Kang ◽  
Helen Nadel ◽  
Christopher Reilly ◽  
...  
Keyword(s):  
Bone Mineral Density ◽  
Duchenne Muscular Dystrophy ◽  
Muscular Dystrophy ◽  
Bone Mineral ◽  
Comparative Effectiveness ◽  
Fragility Fractures ◽  
Estimating Equation ◽  
Treated Group ◽  
Bisphosphonate Therapy ◽  
Mineral Density

The objective of this study was to estimate the comparative effectiveness of bisphosphonate therapy on bone mineral density (BMD) in patients with corticosteroid-treated Duchenne muscular dystrophy (DMD). A retrospective, comparative effectiveness study evaluating changes in BMD and fragility fractures in patients with DMD presenting to British Columbia Children’s Hospital from 1989 to 2017 was conducted. Marginal structural generalized estimating equation models weighted by stabilized inverse-probability of treatment weights were used to estimate the comparative effectiveness of therapy on BMD. Of those treated with bisphosphonates (N = 38), 7 (18.4%), 17 (44.7%), and 14 (36.8%) cases were treated with pamidronate, zoledronic acid, or a combination of both, respectively, while 36 cases of DMD were untreated. Mean age of bisphosphonate initiation was 9.2 (SD 2.7) years. Mean fragility fractures declined from 3.5 to 1.0 following bisphosphonate therapy. Compared to the treated group, the untreated group had an additional 0.63-SD decrease (95% confidence interval [CI]: −1.18, −0.08, P = .026) in total BMD and an additional 1.04-SD decrease (95% CI: −1.74, −0.34; P = .004) in the left hip BMD, but the change in lumbar spine BMD (0.15, 95% CI: −0.36, 0.66; P = .57) was not significant. Bisphosphonate therapy may slow the decline in BMD in boys with corticosteroid-treated DMD compared to untreated counterparts. Total number of fragility fractures decreased following bisphosphonate therapy.

scholarly journals The role of delayed bone age in the evaluation of stature and bone health in glucocorticoid treated patients with Duchenne muscular dystrophy

2019 ◽  
Vol 2019 (1) ◽  
Author(s):  
E. J. Annexstad ◽  
J. Bollerslev ◽  
J. Westvik ◽  
A. G. Myhre ◽  
K. Godang ◽  
...  
Keyword(s):  
Bone Mineral Density ◽  
Duchenne Muscular Dystrophy ◽  
Muscular Dystrophy ◽  
Bone Health ◽  
Bone Age ◽  
Treated Group ◽  
Skeletal Maturation ◽  
Mineral Density ◽  
Duration Of Treatment ◽  
Z Scores

Abstract Background Low bone mineral density and an increased risk of appendicular and vertebral fractures are well-established consequences of Duchenne muscular dystrophy (DMD) and the risk of fractures is exacerbated by long-term glucocorticoid treatment. Monitoring of endocrine and skeletal health and timely intervention in at-risk patients is important in the management of children with DMD. Methods As part of the Norwegian Duchenne muscular dystrophy cohort study, we examined the skeletal maturation of 62 boys less than 18 years old, both currently glucocorticoid treated (n = 44), previously treated (n = 6) and naïve (n = 12). The relationship between bone age, height and bone mineral density (BMD) Z-scores was explored. Results The participants in the glucocorticoid treated group were short in stature and puberty was delayed. Bone age was significantly delayed, and the delay increased with age and duration of treatment. The difference in height between glucocorticoid treated and naïve boys was no longer significant when height was corrected for delayed skeletal maturation. Mean BMD Z-scores fell below − 2 before 12 years of age in the glucocorticoid treated group, with scores significantly correlated with age, duration of treatment and pubertal development. When BMD Z-scores were corrected for by retarded bone age, the increase in BMD Z-scores was significant for all age groups. Conclusion Our results suggest that skeletal maturation should be assessed in the evaluation of short stature and bone health in GC treated boys with DMD, as failing to consider delayed bone age leads to underestimation of BMD Z-scores and potentially overestimation of fracture risk.

Tea flavonoids for bone health: from animals to humans

2016 ◽  
Vol 64 (7) ◽  
pp. 1151-1157 ◽  
Author(s):  
Chwan-Li Shen ◽  
Ming-Chien Chyu
Keyword(s):  
Bone Mineral Density ◽  
Clinical Trials ◽  
Bone Loss ◽  
Bone Mass ◽  
Bone Mineral ◽  
Bone Health ◽  
Fragility Fractures ◽  
Tea Consumption ◽  
Mineral Density ◽  
Risk Of Fracture

Osteoporosis is a skeletal disease characterized by a deterioration of bone mass and bone quality that predisposes an individual to a higher risk of fragility fractures. Emerging evidence has shown that the risk for low bone mass and osteoporosis-related fractures can be reduced by nutritional approaches aiming to improve bone microstructure, bone mineral density, and strength. Tea and its flavonoids, especially those of black tea and green tea, have been suggested to protect against bone loss and to reduce risk of fracture, due to tea's antioxidant and anti-inflammatory properties. Based on the results of animal studies, moderate intake of tea has shown to benefit bone health as shown by mitigation of bone loss and microstructural deterioration as well as improvement of bone strength and quality. Epidemiological studies have reported positive, insignificant, and negative impacts on bone mineral density at multiple skeletal sites and risk of fracture in humans with habitual tea consumption. There are limited human clinical trials that objectively and quantitatively assessed tea consumption and bone efficacy using validated outcome measures in a population at high risk for osteoporosis, along with safety monitoring approach. This review summarizes the current state of knowledge of laboratory animal research, epidemiological observational studies, and clinical trials assessing the skeletal effects of tea and its active flavonoids, along with discussion of relevant future directions in translational research.

scholarly journals Duchenne Muscular Dystrophy - Family in a Crisis

1970 ◽  
pp. 36-39
Author(s):  
M Robed Amin ◽  
Chowdhury Chironjib Borua ◽  
Kaji Shafiqul Alam ◽  
Fazle Rabbi Chowdhury ◽  
Rabiul Jahan Sarkar ◽  
...  
Keyword(s):  
Duchenne Muscular Dystrophy ◽  
Muscular Dystrophy ◽  
Case Series ◽  
Dystrophin Gene ◽  
Muscle Disease ◽  
Motor Neuron Diseases ◽  
Muscle Diseases ◽  
Progressive Muscle Weakness ◽  
Recessive Disorders ◽  
Dystrophin Protein

Progressive muscular weakness with deformity leading to crippled states develop due to musculoskeletal and neurological disorders. Sometimes it is difficult to differentiate between primary muscle disease and neurological disease. But there is some classical presentation of muscle diseases which have its own entity and thus can be clinically differentiated from neurological disorder especially spinal cord and motor neuron diseases. Muscular dystrophy is one of those disorder with distinct clinical features. Muscular dystrophy refers to a group of genetic, hereditary muscle diseases that cause progressive muscle weakness. Most types of MD are multi-system disorders with manifestations in body systems including skeletal system, the heart, gastrointestinal and nervous systems, endocrine glands, skin, eyes and other organs. Duchenne muscular dystrophy (DMD), is inherited in an X-linked recessive pattern, meaning that the mutated gene that causes the disorder is located on the X chromosome, one of the two sex chromosomes, and is thus considered sex-linked. Males are therefore affected by X-linked recessive disorders much more often than females. A characteristic of X-linked inheritance is that fathers cannot pass X-linked traits to their sons. Duchenne muscular dystrophy and Backers muscular dystrophy are caused by mutations of the gene for the dystrophin protein and lead to an overabundance of the enzyme creatine kinase. The dystrophin gene is the largest gene in humans. In this case series a family with three brothers suffering from Duchenne muscular dystrophy is described and review with literature was done.   doi:10.3329/jom.v10i3.2015 J Medicine 2009; 10 (Supplement 1): 36-39

Bone health in Duchenne muscular dystrophy: a workshop report from the meeting in Cincinnati, Ohio, July 8, 2004

2005 ◽  
Vol 15 (1) ◽  
pp. 80-85 ◽  
Author(s):  
W.D. Biggar ◽  
L.K. Bachrach ◽  
R.C. Henderson ◽  
H. Kalkwarf ◽  
H. Plotkin ◽  
...  
Keyword(s):  
Duchenne Muscular Dystrophy ◽  
Muscular Dystrophy ◽  
Bone Health ◽  
Workshop Report
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