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Early detection of lung function decrements in children and adolescents with cystic fibrosis using new reference

Angela Zacharasiewicz1, Sabine Renner2, Flora Haderer2

  • 1Department of Pediatrics and Adolescent Medicine, Wilhelminenspital Vienna, Montleartstrasse 37, 1160, Vienna, Austria. angela.zacharasiewicz@wienkav.at.

Wiener Klinische Wochenschrift
|March 11, 2017
PubMed

Insights

Newer Global Lung Function Initiative (GLI) values for cystic fibrosis (CF) lung function testing result in lower forced expiratory volume in 1s (FEV1)% predicted compared to older Zapletal values. This highlights the need for updated reference standards in CF care.

Area of Science:

  • Pediatric Pulmonology
  • Respiratory Medicine
  • Cystic Fibrosis Research

Background:

  • Accurate interpretation of lung function in children with cystic fibrosis (CF) is crucial for clinical management.
  • Established reference values, such as those by Zapletal et al., have historically guided CF care.
  • Emerging reference standards, like the Global Lung Function Initiative (GLI), aim to provide more current and potentially accurate interpretations.

Purpose of the Study:

  • To investigate the clinical impact of using new Global Lung Function Initiative (GLI) reference values compared to the formerly used Zapletal et al. values for interpreting lung function in children with CF.
  • To determine if discrepancies between these reference sets lead to significantly different clinical outcomes.

Main Methods:

  • Analysis of 3719 lung function measurements from 108 children and adolescents (aged 6-18 years) with CF.
  • Data interpretation using both Zapletal and GLI reference values, analyzed in milliliters (ml) and % predicted (pred.).
  • Comparison of FEV1 and FVC values, including subgroup analysis for Pseudomonas aeruginosa (PA) detection.

Main Results:

  • Application of GLI reference values resulted in significantly lower mean forced expiratory volume in 1s (FEV1)% predicted compared to Zapletal values.
  • A notable percentage of measurements (32%) were misclassified as normal using Zapletal values when compared to GLI.
  • While overall FEV1 and FVC showed no difference between Pseudomonas-positive and negative groups, age-related declines were observed, and small airway obstruction (FEF75) showed significant differences.

Conclusions:

  • The choice of reference values significantly impacts the interpretation of lung function in pediatric CF patients.
  • Using older reference values may lead to a false sense of reassurance regarding lung function status.
  • Standardizing the use of the most current and appropriate reference values, such as GLI, is essential for optimal clinical decision-making in CF management.

Keywords:
ChildrenCystic fibrosisLung functionReference valuesSpirometry

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