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Lung clearance index at 4 years predicts subsequent lung function in children with cystic fibrosis

Paul Aurora1, Sanja Stanojevic, Angie Wade

  • 1Portex Respiratory Unit, UCL Institute of Child Health, 30 Guilford St., London WC1N 1EH, UK. p.aurora@ich.ucl.ac.uk.

American Journal of Respiratory and Critical Care Medicine
|October 12, 2010
PubMed

Insights

Multiple-breath washout (MBW) detects early lung disease in children with cystic fibrosis (CF). Abnormal lung clearance index (LCI) in preschoolers predicts future lung function decline, aiding early intervention.

Area of Science:

  • Pediatric Pulmonology
  • Respiratory Medicine

Background:

  • Improved life expectancy in cystic fibrosis (CF) necessitates early lung disease detection in young children.
  • Traditional lung function markers are often normal in preschool-aged children with CF.
  • There is a critical need for sensitive biomarkers to identify early lung disease and track progression in young CF patients.

Purpose of the Study:

  • To evaluate if multiple-breath washout (MBW) results in preschool-aged children with CF can predict future abnormal lung function.
  • To establish the predictive value of MBW parameters for long-term respiratory health in pediatric CF.

Main Methods:

  • Preschool children (3-5 years) with CF and healthy controls underwent spirometry and MBW.
  • Testing was repeated in early school age (6-10 years).
  • Primary outcomes included forced expiratory volume in 1 second (FEV1) from spirometry and lung clearance index (LCI) from MBW.

Main Results:

  • 73% of preschool children with CF had an abnormal LCI, compared to only 5 with abnormal FEV1.
  • Preschool LCI demonstrated a high positive predictive value (94%) for abnormal school-age lung function.
  • Preschool FEV1 had a 100% positive predictive value but a low negative predictive value (25%) for predicting later abnormalities.

Conclusions:

  • An abnormal preschool LCI is a strong predictor of subsequent lung function abnormalities in children with CF.
  • A normal preschool LCI typically indicates sustained normal lung function.
  • Multiple-breath washout (MBW) shows significant potential as a clinical and research outcome measure for young children with CF.
Abstract

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