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Bone mineral density is related to lung function outcomes in young people with cystic fibrosis-A retrospective study

Nathan Smith1, Angelina Lim1,2, Matthew Yap1

  • 1Murdoch Childrens Research Institute, Royal Children's Hospital, Victoria, Australia.

Pediatric Pulmonology
|October 31, 2017
PubMed

Insights

Children with cystic fibrosis (CF) and poor lung function or nutrition have lower bone mineral density (BMD). This suggests targeted bone health screening for CF patients with the most severe clinical status.

Area of Science:

  • Pediatric Endocrinology
  • Pulmonology
  • Genetics

Background:

  • Medical advancements have increased survival in cystic fibrosis (CF), leading to more long-term complications like low bone mineral density (BMD).
  • Low BMD in CF patients increases the risk of fractures later in life.
  • Understanding the factors influencing BMD in CF is crucial for proactive management.

Purpose of the Study:

  • To investigate the relationship between BMD and lung function, nutritional status, and genetic factors in children with CF.
  • To identify key clinical indicators associated with reduced BMD in this population.

Main Methods:

  • Retrospective audit of 202 children with CF from August 2000 to January 2016.
  • Analysis of dual-energy X-ray absorptiometry (DXA) data to assess BMD Z-scores.
  • Correlation of BMD Z-scores with clinical status, nutritional parameters, and CF genotype.

Main Results:

  • A strong association was found between the severity of lung disease and BMD Z-scores (P < 0.0001).
  • Nutritional status was also significantly correlated with BMD Z-scores (P < 0.05).
  • These findings highlight the interplay between pulmonary health, nutrition, and bone density in CF.

Conclusions:

  • This study provides substantial evidence linking the severity of pulmonary disease to BMD in cystic fibrosis patients.
  • Screening guidelines for bone health in children with CF should prioritize individuals with the most compromised clinical status.
  • Early identification and intervention for low BMD can mitigate fracture risk in this vulnerable population.
Abstract

Keywords:
cystic fibrosisepidemiologyevidence-based medicine and outcomesimaging

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