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Guide to bone health and disease in cystic fibrosis

Robert M Aris1, Peter A Merkel, Laura K Bachrach

  • 1CB 7020, 4131 Bioinformatics, University of North Carolina, Chapel Hill, North Carolina 27599-7524, USA. aris@med.unc.edu

The Journal of Clinical Endocrinology and Metabolism
|December 23, 2004
PubMed

Insights

Cystic fibrosis (CF) patients face significant bone disease risks, including low bone density and fractures. Addressing multiple risk factors is crucial for managing bone health in adults with CF.

Area of Science:

  • Bone biology and genetics
  • Pulmonary medicine
  • Endocrinology

Background:

  • Cystic fibrosis (CF) is a prevalent genetic disorder primarily affecting Caucasian populations, leading to respiratory failure.
  • Increased lifespan in CF patients has revealed bone disease as a common complication, with 50-75% of adults experiencing low bone density and fractures.
  • The rising number of adult CF survivors necessitates a focus on managing chronic complications like bone disease.

Framework:

  • This review summarizes the Cystic Fibrosis Foundation's 2002 consensus conference on CF-related bone disease.
  • It addresses the pathogenesis, diagnosis, and treatment of bone complications in cystic fibrosis.
  • The goal was to establish practice guidelines for optimizing bone health in CF patients.

Implementation:

  • Key risk factors for poor bone health in CF include pancreatic insufficiency affecting vitamin absorption, hormonal imbalances, chronic infections, physical inactivity, and corticosteroid use.
  • Effective management requires a multifactorial approach targeting these specific risk factors.
  • Early diagnosis and intervention are vital for preventing fractures and improving long-term outcomes.

Implications:

  • Optimizing bone health is critical for the quality of life and long-term survival of individuals with cystic fibrosis.
  • Developing and implementing evidence-based guidelines can significantly reduce the burden of bone disease in CF.
  • Further research into the complex interplay of factors affecting bone metabolism in CF is warranted.

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