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Review
. 2013 Dec;20(6):547-52.
doi: 10.1097/01.med.0000436191.87727.ec.

Cystic fibrosis-related bone disease: insights into a growing problem

Michael S Stalvey  1 , Gregory A Clines
Affiliations
Review

Cystic fibrosis-related bone disease: insights into a growing problem

Michael S Stalvey et al. Curr Opin Endocrinol Diabetes Obes. 2013 Dec.

Abstract

Purpose of review: This review will describe the clinical significance, pathogenesis and treatment of cystic fibrosis related bone disease (CFBD).

Recent findings: CFBD continues to increase as the life expectancy of individuals with cystic fibrosis increases. According to clinical guidelines, individuals with cystic fibrosis should be initially screened at the age of 18 years via dual-energy x-ray absorptiometry, if not done so previously. The underlying pathogenesis of CFBD appears to be multifactorial, but increasing data imply a direct impact by the cystic fibrosis transmembrane conductance regulator (CFTR). CFTR deficiency and/or dysfunction impair osteoblast activity and differentiation, and indirectly promote osteoclast formation. Unfortunately, once diagnosed with CFBD, few cystic fibrosis tested medical therapies exist.

Summary: CFBD is an increasingly recognized complication that has a significant impact on the overall health of the individual. Recommendations to identify patients with cystic fibrosis who are at risk for fracture using dual-energy x-ray absorptiometry have been established. Therapeutic agents directly studied in patients with cystic fibrosis are limited to bisphosphonates, although other potential treatment agents exist. Finally, an improved understanding of the pathologic mechanisms will aid in the study and development of therapies.

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Figures

Fig. 1
Fig. 1
Cystic Fibrosis-Related Bone Disease (CFBD) origniates from multiple systemic and direct factors associated with CF.
Fig. 2
Fig. 2
The dynamic status of bone turnover is uncoupled in CF. This results in a decreased bone formation, with an increased bone resorption state.
See this image and copyright information in PMC

References

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