Impact of cystic fibrosis on bone health

Curr Opin Pulm Med. 2010 Aug 25.



Impact of cystic fibrosis on bone health.

Haworth CS.
Adult Cystic Fibrosis Centre, Papworth Hospital, Cambridge, UK.



Abstract

PURPOSE OF REVIEW: This review summarizes recently published data on the epidemiology, pathophysiology and treatment of cystic fibrosis (CF)-related low bone mineral density (BMD).



RECENT FINDINGS: A systematic literature review reports that the pooled prevalence of osteoporosis in adults with CF is 23.5% [95% confidence interval (CI) 16.6-31.0] and the pooled prevalences of radiologically confirmed vertebral and nonvertebral fractures are 14% (95% CI 7.8-21.7) and 19.7% (95% CI 6.0-38.8), respectively. Recent data suggest that the cystic fibrosis transmembrane conductance regulator (CFTR) is expressed in bone cells and that CFTR dysfunction affects bone cell activity. The secondary effects of CFTR dysfunction also influence bone metabolism. For example, bone resorption increases during CF pulmonary exacerbations due to the stimulatory effects of proinflammatory cytokines on osteoclast activity. Bisphosphonates inhibit osteoclastic bone resorption and recent data show that both oral and intravenous bisphosphonates improve BMD in patients with CF.



SUMMARY: CF-related low BMD is a common but treatable complication of CF.

High rates of fracture among CFer's

Calcif Tissue Int. 2009 Dec 1.

The Prevalence of Osteoporosis, Osteopenia, and Fractures Among Adults with Cystic Fibrosis: A Systematic Literature Review with Meta-Analysis.

Paccou J, Zeboulon N, Combescure C, Gossec L, Cortet B.

Département Universitaire de Rhumatologie, Centre Hospitalier et Universitaire, Hôpital Roger Salengro, Lille, France, julienpaccou@yahoo.fr.

Observational studies have indicated a high but heterogeneous prevalence of low bone mineral density for adult patients with cystic fibrosis. Fracture complications were also described. The objective of this study was to determine the prevalence of osteoporosis, osteopenia, and fractures among adult patients with cystic fibrosis.

A systematic literature review was conducted using electronic databases. The keywords used were "cystic fibrosis [MeSH] AND bone density." Original studies were eligible if they reported the prevalence of osteoporosis and/or osteopenia and/or fractures in adult patients with cystic fibrosis. A meta-analysis of pooled proportions was performed. Heterogeneity was tested with the Cochran Q statistic, and in the case of heterogeneity a random effect model was used. Of 117 studies, 12 were selected, i.e., that represented a total of 1055 patients. Mean age ranged from 18.5 to 32 years (median: 28.2 years). Mean body mass index ranged from 19.9 to 22.4 (median: 20.7); 53.8% were men.

The pooled prevalence of osteoporosis in adults with cystic fibrosis was 23.5% (95% CI, 16.6-31.0). The pooled prevalence of osteopenia was 38% (95% CI, 28.2-48.3). The pooled prevalences of radiological vertebral fractures and nonvertebral fractures were 14% (95% CI, 7.8-21.7) and 19.7% (95% CI, 6.0-38.8), respectively.

In conclusion, this systematic literature review with meta-analysis emphasized the high prevalence of osteopenia and osteoporosis in young adults with cystic fibrosis. The prevalence of fracture was also high.