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

Michael S Stalvey1, Gregory A Clines

  • 1aDepartment of Pediatrics bDepartment of Medicine, University of Alabama at Birmingham cVeterans Administration Medical Center, Birmingham, Alabama, USA.

Current Opinion in Endocrinology, Diabetes, and Obesity
|January 29, 2014
PubMed

Insights

Cystic fibrosis related bone disease (CFBD) is rising with increased lifespan. Early screening and understanding CFTR

Area of Science:

  • Bone Metabolism and Disease
  • Genetics and Molecular Biology
  • Pulmonology and Respiratory Medicine

Background:

  • Cystic fibrosis related bone disease (CFBD) is an emerging complication in individuals with cystic fibrosis (CF).
  • Increased life expectancy in CF patients contributes to a higher prevalence of CFBD.
  • CFBD significantly impacts the overall health and quality of life for individuals with CF.

Purpose of the Study:

  • To review the clinical significance of CFBD.
  • To elucidate the pathogenesis of CFBD, focusing on the role of the cystic fibrosis transmembrane conductance regulator (CFTR).
  • To discuss current and potential treatment strategies for CFBD.

Main Methods:

  • This is a review article, synthesizing existing literature on CFBD.
  • Data were gathered from clinical guidelines and research studies on CF pathogenesis and bone health.
  • Analysis focused on the impact of CFTR dysfunction on bone metabolism.

Main Results:

  • CFBD prevalence is increasing in the aging CF population.
  • Dual-energy X-ray absorptiometry (DXA) is recommended for screening CF patients aged 18 and older.
  • Evidence suggests CFTR deficiency directly impairs osteoblast function and promotes osteoclast activity, contributing to CFBD pathogenesis.

Conclusions:

  • CFBD is a significant and growing concern in cystic fibrosis care.
  • Established screening protocols using DXA can identify at-risk individuals.
  • Current therapeutic options for CFBD are limited, with bisphosphonates being the primary studied agent, highlighting the need for further research into novel treatments based on improved understanding of CFBD pathology.
Abstract

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