PATHOGENESIS OF OSTEOPOROSIS IN YOUNG CHILDREN WITH CYSTIC FIBROSIS

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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. Osteoporosis, skeletal pain, and fractures are frequent sequelae of cystic fibrosis (CF). The pathogenesis of bone disease in individuals with CF is complex, resulting both from processes that decrease bone formation and that increase bone resorption. Bone turnover in adults with CF has been shown to be abnormal with increased bone resorption yet normal bone formation. NO study as comprehensively assessed vitamin D metabolism in young children with CF. This study will compare children with CF to their unaffected siblings. Siblings were chosen as the controls in order to provide a comparison group for bone turnover measures and densitometric measurements and also to explore if there are effects of heterozygous CF transmembrane receptor mutations on bone mineral accrual. Assessment of bone mass and bone metabolism in prepubertal children with CF will allow determination of the role of these factors in the development of osteoporosis in these individuals. This will enable development of rational management and prophylactic interventions and therapies.