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Cystic fibrosis-related bone disease explored using a four step algorithm.

Denise S K Brookes1, Julie N Briody2, Craig F Munns3

  • 1The University of Queensland, Children's Nutrition Research Centre, Queensland Children's Medical Research Institute, Brisbane, Australia; The University of Queensland, Children's Nutrition Research Centre, School of Medicine, Brisbane, Australia.

Journal of Cystic Fibrosis : Official Journal of the European Cystic Fibrosis Society
|August 30, 2014
PubMed
Summary

Young individuals with cystic fibrosis (CF) may have suboptimal bone accrual. A new algorithm using dual-energy X-ray absorptiometry (DXA) data helps interpret bone health by considering body size and lean tissue mass (LTM).

Keywords:
AlgorithmBMCCystic fibrosisDXAHeightLTM

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Area of Science:

  • Pediatric Bone Health
  • Cystic Fibrosis Research
  • Body Composition Analysis

Background:

  • Suboptimal bone accrual in young individuals with cystic fibrosis (CF) may lead to premature CF-related bone disease.
  • Dual-energy X-ray absorptiometry (DXA) is a standard measure for bone health, but its interpretation is limited by body size and lean tissue mass (LTM) influences.
  • Understanding these influences is crucial for accurate assessment of bone health in pediatric CF patients.

Purpose of the Study:

  • To evaluate the influence of body size and LTM on bone mineral content (BMC) measurements in children and adolescents with CF.
  • To develop and apply a novel algorithm for a more accurate interpretation of DXA-derived bone data in the context of CF.
  • To differentiate the impact of short stature versus LTM on reduced BMC in CF.

Main Methods:

  • Comparison of total body DXA measurements (BMC, LTM) between 53 individuals with CF and 53 sex-matched controls (ages 7.00-17.99 years).
  • Calculation of BMC, height, and LTM in relation to height and BMC Z-scores.
  • Utilization of a 4-step algorithm to analyze the relationship between BMC, LTM, and stature.

Main Results:

  • Pubertal females with CF exhibited lower total body BMC for their age (p=0.02).
  • Pre-pubertal males (p=0.05) and pubertal females (p=0.03) with CF were shorter than controls.
  • Pubertal females with CF demonstrated significantly less total body BMC relative to their LTM (p=0.01).

Conclusions:

  • The developed algorithm indicated that pre-pubertal low total body BMC in CF is primarily linked to short stature.
  • Lean tissue mass (LTM) was found to be appropriate for body size across the studied CF cohort.
  • Pubertal females with CF showed reduced total body BMC in relation to their LTM, highlighting the importance of body composition in bone health assessment.
  • Further controlled trials are necessary to refine the clinical interpretation of DXA data for CF-related bone disease, incorporating patient size and body composition.