Interpretation of lung function in infants and young children with cystic fibrosis

Respirology. 2014 Aug;19(6):792-9. doi: 10.1111/resp.12329. Epub 2014 Jun 19.


The last decade has seen a significant advance in understanding about early lung disease in cystic fibrosis (CF). As studies that have measured lung function in preschool years are conducted in association with surveillance of infection, inflammation and early structural changes, and emerging longitudinal data become available, a better insight into the very early onset and nature of such lung disease is emerging. Interventions during the preschool years are increasingly viewed as being crucial to delaying and minimizing disease progression as this is the most important period of postnatal life in terms of lung development and airway remodelling. Lung function measurement in CF is potentially an important assessment tool and is used in routine clinical practice in several centres already. Results of studies from lung function tests that, on the basis of their underpinning physiology, are viewed as being best suited currently for the early detection of lung disease in CF are reviewed.

Keywords: cystic fibrosis; infection and inflammation; paediatric lung disease; respiratory function test; respiratory structure and function.

Publication types

  • Review

MeSH terms

  • Age Factors
  • Breath Tests
  • Child
  • Child, Preschool
  • Cystic Fibrosis / physiopathology*
  • Forced Expiratory Volume / physiology
  • Humans
  • Infant
  • Infant, Newborn
  • Lung / physiopathology*
  • Respiratory Function Tests*
  • Spirometry