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Pulmonary Function Tests in the Evaluation of Early Lung Disease in Cystic Fibrosis

Katarzyna Walicka-Serzysko1,2, Magdalena Postek1,2, Urszula Borawska-Kowalczyk1,2

  • 1Cystic Fibrosis Department, Institute of Mother and Child, 01-211 Warsaw, Poland.

Journal of Clinical Medicine
|July 29, 2023
PubMed

Insights

Multiple breath nitrogen washout (MBNW) and impulse oscillometry (IOS) are superior to spirometry for detecting early lung disease in children with cystic fibrosis (CF). The lung clearance index (LCI) is a more sensitive measure than FEV1.

Area of Science:

  • Pediatric Pulmonology
  • Respiratory Medicine
  • Cystic Fibrosis Research

Background:

  • Early detection of respiratory dysfunction is critical in pediatric cystic fibrosis (CF) management.
  • Conventional pulmonary function tests (PFTs) may not adequately capture early lung disease in CF.
  • Novel techniques are needed to assess subtle changes in lung function.

Purpose of the Study:

  • To evaluate the effectiveness of multiple breath nitrogen washout (MBNW) and impulse oscillometry (IOS) in assessing early lung disease in children with CF.
  • To compare MBNW and IOS with conventional spirometry and body plethysmography.
  • To identify sensitive biomarkers for early functional abnormalities.

Main Methods:

  • Prospective study involving 69 children with CF aged 7-18 years.
  • Pulmonary function tests including MBNW, IOS, spirometry, and body plethysmography were performed.
  • Nutritional status, microbiological data, pulmonary exacerbations (PExs), and health-related quality of life (HRQoL) were also assessed.

Main Results:

  • Spirometry showed normal lung function in 49.3%, mild disease in 36.2%, and moderate disease in 14.5% of patients.
  • An elevated lung clearance index (LCI > 6.98), a measure from MBNW, was found in 85% of children with normal spirometry (FEV1 ≥ 90%pred).
  • Pseudomonas aeruginosa infection and a higher number of PExs correlated with poorer PFT results.

Conclusions:

  • MBNW and IOS are more sensitive than conventional techniques for evaluating early lung disease in pediatric CF.
  • The lung clearance index (LCI) is a more effective parameter than forced expiratory volume in 1 second (FEV1) for detecting functional abnormalities in school-aged children with CF.
Abstract

Keywords:
CFQ-RHRQOLcystic fibrosisearly lung diseaseimpulse oscillometrylung clearance indexpulmonary exacerbationpulmonary function testspirometry

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