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Infant Lung Function in Cystic Fibrosis: A Real-World Study

Michele Arigliani1,2, Sidrah Chaudhry1, Rossa Brugha1,2

  • 1Paediatric Respiratory Unit, Great Ormond Street Hospital for Children NHS Foundation Trust, London, UK.

Pediatric Pulmonology
|May 6, 2025
PubMed

Insights

Infant lung function abnormalities are common in cystic fibrosis but clinical management primarily relies on symptoms, not lung function tests. Real-world data show management changes are infrequent even with abnormal lung function.

Area of Science:

  • Pediatric Pulmonology
  • Cystic Fibrosis Research
  • Infant Lung Function Assessment

Background:

  • Lung function abnormalities are frequently observed in infants with cystic fibrosis (IwCF).
  • Limited real-world data exists on the clinical significance of these abnormalities in IwCF.
  • Understanding early lung function changes is crucial for timely intervention in IwCF.

Purpose of the Study:

  • To investigate the prevalence of lung function abnormalities in infants with cystic fibrosis.
  • To assess the correlation between lung function test results and clinical management decisions in IwCF.
  • To analyze the impact of microbiology and clinical findings on treatment adjustments in IwCF.

Main Methods:

  • Retrospective analysis of infant lung function data from IwCF (2012-2018) at a single center.
  • Lung function tests (SF6 Lung Clearance Index [LCI], FRC, FEV0.5) performed at 3 months, 1 year, and 2 years.
  • Analysis of microbiology, antibiotic prescriptions, and clinical management changes around lung function assessments.

Main Results:

  • Lung function abnormalities (elevated zLCI) were present in 31% of 3-month-olds, 28% of 1-year-olds, and 19% of 2-year-olds.
  • In cases with positive microbiology or abnormal chest findings, 100% of those with abnormal lung function and 86% with normal lung function received antibiotic prescriptions or management changes.
  • Management changes occurred in only 12% of cases with abnormal lung function but normal clinical findings.

Conclusions:

  • Clinical management in infants with cystic fibrosis is predominantly driven by clinical findings rather than solely by lung function test results.
  • Abnormal lung function (elevated FRC or LCI) had a marginal influence on clinical management decisions in this real-world cohort.
  • Further research is needed to optimize the integration of lung function data into clinical decision-making for IwCF.
Abstract

Keywords:
cystic fibrosismultiple breath washoutpediatricsreal‐world evidencerespiratory function tests

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