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

Stalvey, Michael S.a; Clines, Gregory A.b,c

Current Opinion in Endocrinology, Diabetes and Obesity: December 2013 - Volume 20 - Issue 6 - p 547–552
doi: 10.1097/01.med.0000436191.87727.ec
PARATHYROIDS, BONE AND MINERAL METABOLISM: Edited by Vin Tangpricha

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.

aDepartment of Pediatrics

bDepartment of Medicine, University of Alabama at Birmingham

cVeterans Administration Medical Center, Birmingham, Alabama, USA

Correspondence to Michael S. Stalvey, MD, Department of Pediatrics, Division of Pediatric Endocrinology, University of Alabama at Birmingham, The Children's Hospital, CPPII M30, 1600 7th Ave S., Birmingham, AL 35233-1711, USA. Tel: +1 205 638 9107; e-mail: mstalvey@peds.uab.edu

© 2013 Wolters Kluwer Health | Lippincott Williams & Wilkins