This subproject is one of many research subprojects utilizing the resources provided by a Center grant funded by NIH/NCRR. The subproject and investigator (PI) may have received primary funding from another NIH source, and thus could be represented in other CRISP entries. The institution listed is for the Center, which is not necessarily the institution for the investigator. Duchene muscular dystrophy (DMD) is a severe genetic disorder that often leads to loss of ambulation by age 12 and death by age 20-25. Disuse osteopenia has been described when weight bearing stimulus, necessary for bone remodeling especially in growth, is missing. Decreased muscle strength and corticosteroid use also appear to be factors affecting bone mineral density (BMD) in children with DMD. The standard of care is based on long-term use of corticosteriods. While corticosteroid therapy improves and prolongs muscle function, it also appears to increase the risk of bone fractures. 21-40% of the children with DMD will experience a long bone fracture with risk increasing with age. Most of these fractures occur with minimal or no trauma. The objective of this study is to collect cross sectional data of lumbar spine and femur DXA and tibia pQCT BMD, vitamin D and bone marker levels, dietary calcium intake, and functional status (according to a functional scale of 1-8) of DMD children with or without chronic glucocorticoids therapy. Fracture history and prevalence in DMD will be correlated with lumbar spine, femur and tibia bone mineral densities, steroid use, and mobility status. The overall goal is to determine whether glucocorticoid-induced bone loss in children with DMD creates conditions that merit pharmacologic intervention.
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