Cystic Fibrosis Research Today is a free monthly online journal that collates and summarizes the latest research about Cystic Fibrosis, including details on symptoms, genetics, treatment, information.

Reduced bone density in cystic fibrosis: DeltaF508 mutation is an independent risk factor.

King SJ, Topliss DJ, Kotsimbos T, Nyulasi IB, Bailey M, Ebeling PR, Wilson JW

Nutrition Dept, Alfred Hospital, Commercial Road, Melbourne, Victoria 3004, Australia. susannah.king@med.monash.edu.au

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-alpha, and interleukin-6 and -1beta. 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 <-2.0 were found in 17% of subjects. Subjects who were homozygous or heterozygous for the DeltaF508 mutation exhibited significantly lower Z-scores than those with no DeltaF508 allele. Multiple linear regression showed that the DeltaF508 genotype and male sex were independently associated with lower BMD at both sites. Other factors also independently associated with lower BMD included malnutrition, lower 25-hydroxyvitamin D level, lower fat-free mass and lower FEV1 (% pred). In conclusion, reduced bone mineral density in cystic fibrosis is associated with a number of factors, including DeltaF508 genotype, male sex, greater lung disease severity and malnutrition.

Published 10 January 2005 in Eur Respir J, 25(1): 54-61.
Full-text of this article is available online (may require subscription).

© 2004-2008 Cystic Fibrosis Research Today. All Rights Reserved.