Lung function testing in infants with cystic fibrosis: lessons from the past and future directions

Pediatr Pulmonol. 2001 Sep;32(3):228-45. doi: 10.1002/ppul.1113.


Despite the increasing awareness of the need to identify early pulmonary changes in cystic fibrosis (CF) noninvasively, the role of lung function testing in infancy and early childhood remains less clear than in older children with CF. The aim of this review is to summarize available data, discuss the information gained from these publications, and put this information into perspective with more recent developments of lung function testing in both infants and older children with CF. While some of the available data have been the foundation of the current level of understanding of respiratory physiology in CF, interpretation of other data has been hampered by differences between centers with regard to the methods and equipment used, patient selection, small number of subjects, and lack of appropriate reference data. A structured multicenter approach based on recently published recommendations for the measurement of lung function in infancy, together with pursuit of recent developments such as assessment of raised lung volume flow volume curves and ventilation inhomogeneity may help to more effectively utilize lung function tests in infants in the future.

Publication types

  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Clinical Trials as Topic
  • Cystic Fibrosis / diagnosis*
  • Cystic Fibrosis / pathology
  • Diagnosis, Differential
  • Humans
  • Infant
  • Infant Welfare*
  • Infant, Newborn
  • Longitudinal Studies
  • Lung / growth & development
  • Lung / physiology*
  • Prognosis
  • Respiratory Function Tests