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J Pediatr. 1996 Jan;128(1):28-34.

Bone density in children and adolescents with cystic fibrosis.

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Department of Orthopedics, University of North Carolina, Chapel Hill 27599-7055, USA.



To assess bone mineralization in children and adolescents with cystic fibrosis.


A cross-sectional, observational study of bone mineral density (BMD) in the lumbar vertebrae and the proximal femur of 62 patients aged 4.9 to 17.8 years (mean, 10.7 years). The age-normalized BMD findings (z scores) were correlated with multiple variables, including measures of pulmonary disease, nutritional status and growth, genotype, calcium intake, and serum 25-hydroxyvitamin D and 1,25-dihydroxyvitamin D levels.


The mean BMD z score was -1.03 +/- 0.14 (+/- SE) in the lumbar vertebrae and -0.71 +/- 0.17 in the proximal femur. The BMD in this age range declined relative to normal values at a rapid rate of roughly 1 SD every 6 to 8 years. The BMD z scores correlated well with multiple measures of disease severity, particularly weight and forced expiratory volume in 1 second.


Significant osteoporosis in adults with CF results at least in part from a failure to accumulate bone mineral at a normal rate during skeletal growth and development. The cause of this is likely multifactorial. With increasing longevity, the skeletal consequences of CF become an important consideration.

[Indexed for MEDLINE]

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