Reduced bone density in cystic fibrosis: ΔF508 mutation is an independent risk factor

Abstract

The aim of this cross-sectional study was to determine the prevalence and identify determinants of reduced bone mineral density (BMD) in adults with cystic fibrosis (CF). Adults (88) with CF (mean ± SD age 29.9 ± 7.7 yrs; forced expiratory volume in one second (FEV1) 58.2 ± 21.5% of the predicted value) were studied. BMD at the lumbar spine (LS) and femoral neck (FN) and body composition were measured using dual-energy X-ray absorptiometry. Blood and urine were analysed for hormones, bone turnover markers, and the cytokines tumour necrosis factor-α, and interleukin-6 and -1β. FEV1 (% pred); CF genotype; malnutrition; history of growth, development or weight gain delays; and corticosteroid use were analysed. BMD Z-scores were -0.58 ± 1.30 (mean ± SD) at the LS and -0.24 ± 1.19 at the FN. Z-scores of