足と足首の臨床研究

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Clinical Recommendations to Improve Bone Health in Children with Duchenne Muscular Dystrophy

Jawn L

The X-linked recessive disease Duchenne muscular dystrophy (DMD) causes progressive muscle weakness that leads to eventual loss of ambulation and early death. Enhancing muscle strength, extending ambulation, and maintaining pulmonary function are all benefits of the approved corticosteroid therapy. However, the chronic use of corticosteroids has an osteoporotic effect that worsens the DMD-related reduced bone mass and increases the risk of long bone and vertebral fragility fractures. These severe consequences can have an impact on survival and negatively affect quality of life. This analysis discusses the current clinical concerns around bone health and approaches for bone health screening in DMD. Diagnostic procedures, such as dual energy X-ray absorptiometry (DXA), densitometric lateral spinal imaging, and biochemical markers of bone turnover and bone mineral density, as well as therapies to improve bone health in DMD patients, are reviewed. Bisphosphonate therapy offers a way to boost these kids' bone mass; both oral and intravenous bisphosphonates have been used successfully, though therapy is normally saved for kids with fractures and/or bone pain who have low bone mass according to DXA.