Vertebral Fractures in Boys with Duchenne Muscular Dystrophy

Je, Bothwell; Ke, Gordon; Jm, Dooley; MacSween, Judith; Cummings, Elizabeth A.; Salisbury, Sonia

doi:10.1177/000992280304200408

Cited by 103 publications

(65 citation statements)

References 8 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…7,31,33,41,50 Bone mineral density must be followed by DXA; measurement of vitamin D level is useful, and any history of fracture (frequency, site, and trauma) should be recorded. 12 In general, the beneficial response to steroids in functional tests is particularly evident in younger children (Fig. 7).…”

Section: Long-term Use Of Steroidsmentioning

confidence: 93%

“…Vertebral fractures are rarely seen in DMD patients who are not treated with steroids. 12,31,33,51 Although several techniques exist to measure bone mineral density, there are problems with interpretation of the results and, specifically, the interpretation of what a single finding in different bones (e.g., spine, hip) might mean to an individual patient.…”

Section: Side-effect Monitoringmentioning

confidence: 99%

See 1 more Smart Citation

The role of corticosteroids in muscular dystrophy: A critical appraisal

2007

View full text Add to dashboard Cite

Over the years various steroid trials have been conducted in Duchenne muscular dystrophy (DMD). In children who are still able to walk as well as in those who are wheelchair-bound, corticosteroids have been found to stabilize muscle strength for a period of time. Controlled clinical observations have shown that some boys remain ambulatory for years longer than reported in natural history data. The two main steroids used are prednisone/prednisolone and deflazacort. They are probably equally effective in stabilizing muscle strength but may have different side-effect profiles; for instance, deflazacort causes less weight gain. The exact mechanism by which steroids slow the dystrophic process is under investigation. DMD children treated long term also seem to develop other complications of the condition less frequently. For instance, they develop respiratory insufficiency later and have fewer cardiac symptoms. The therapeutic value of corticosteroids is limited, but these drugs represent the best treatment option currently available.

show abstract

Section: Long-term Use Of Steroidsmentioning

confidence: 93%

Section: Side-effect Monitoringmentioning

confidence: 99%

The role of corticosteroids in muscular dystrophy: A critical appraisal

2007

View full text Add to dashboard Cite

show abstract

“…GC therapy, used to improve quality of life and ambulation in DMD, exerts additional skeletal toxicity. Studies have shown reduced height-adjusted BMD Z-scores and a long bone fracture prevalence of 20-44% (64, 65) prior to starting GC and an increased VF prevalence of 19-32% following GC exposure (64,66,67). Although no difference in long bone fractures was noted during GC therapy, a significant decline in BMD occurred as the patients lost their ability to walk (Fig.…”

Section: Immobility Induced Osteoporosismentioning

confidence: 98%

ENDOCRINOLOGY AND ADOLESCENCE: Osteoporosis in children: diagnosis and management

Saraff

Högler

2015

European Journal of Endocrinology

View full text Add to dashboard Cite

Osteoporosis in children can be primary or secondary due to chronic disease. Awareness among paediatricians is vital to identify patients at risk of developing osteoporosis. Previous fractures and backaches are clinical predictors, and low cortical thickness and low bone density are radiological predictors of fractures. Osteogenesis Imperfecta (OI) is a rare disease and should be managed in tertiary paediatric units with the necessary multidisciplinary expertise. Modern OI management focuses on functional outcomes rather than just improving bone mineral density. While therapy for OI has improved tremendously over the last few decades, this chronic genetic condition has some unpreventable, poorly treatable and disabling complications. In children at risk of secondary osteoporosis, a high degree of suspicion needs to be exercised. In affected children, further weakening of bone should be avoided by minimising exposure to osteotoxic medication and optimising nutrition including calcium and vitamin D. Early intervention is paramount. However, it is important to identify patient groups in whom spontaneous vertebral reshaping and resolution of symptoms occur to avoid unnecessary treatment. Bisphosphonate therapy remains the pharmacological treatment of choice in both primary and secondary osteoporosis in children, despite limited evidence for its use in the latter. The duration and intensity of treatment remain a concern for longterm safety. Various new potent antiresorptive agents are being studied, but more urgently required are studies using anabolic medications that stimulate bone formation. More research is required to bridge the gaps in the evidence for management of paediatric osteoporosis.

show abstract

“…[140] Vertebral fractures have been detected in 32-40% of boys on long-term corticosteroids, although many are incidental and often not a reason to discontinue treatment. [110,151] Long-bone fractures are also twice as likely compared to steroid-naïve patients. [110] Growth suppression is also seen after long-term corticosteroid treatment.…”

Section: Corticosteroid Therapymentioning

confidence: 99%

Duchenne muscular dystrophy

Yiu

Kornberg

2015

J Paediatrics Child Health

272

242

View full text Add to dashboard Cite

Duchenne muscular dystrophy (DMD), an X-linked disorder, is the most common muscular dystrophy in children, presenting in early childhood and characterized by proximal muscle weakness and calf hypertrophy in affected boys. Patients usually become wheelchair-bound by the age of 12 years, and die of cardiorespiratory complications in their late teens to early twenties. Advances in the management of DMD, including treatment with corticosteroids and the use of intermittent positive pressure ventilation have provided improvements in function, ambulation, quality of life and life expectancy, although novel therapies still aim to provide a cure for this devastating disorder. The clinical features, investigations, and management of DMD are reviewed, as well as the latest in some of the novel therapies.

show abstract

Vertebral Fractures in Boys with Duchenne Muscular Dystrophy

Cited by 103 publications

References 8 publications

The role of corticosteroids in muscular dystrophy: A critical appraisal

The role of corticosteroids in muscular dystrophy: A critical appraisal

ENDOCRINOLOGY AND ADOLESCENCE: Osteoporosis in children: diagnosis and management

Duchenne muscular dystrophy

Contact Info

Product

Resources

About