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Lung clearance index-triggered intervention in children with cystic fibrosis - A randomised pilot study

Christian Voldby1, Kent Green1, Thomas Kongstad1

  • 1CF Centre Copenhagen, Paediatric Pulmonary Service, Department of Paediatrics and Adolescent Medicine, Copenhagen University Hospital, Rigshospitalet, Blegdamsvej 9, Copenhagen, Denmark.

Journal of Cystic Fibrosis : Official Journal of the European Cystic Fibrosis Society
|June 25, 2020
PubMed

Insights

Triggering bronchoalveolar lavage (BAL) with lung clearance index (LCI) increases did not improve clinical outcomes in children with cystic fibrosis (CF). This study suggests LCI-triggered BAL is not beneficial for CF patients.

Area of Science:

  • Pediatric Pulmonology
  • Infectious Diseases in Cystic Fibrosis
  • Respiratory Medicine

Background:

  • Cystic Fibrosis (CF) management often involves monitoring lung function and treating infections.
  • Lung clearance index (LCI) is a sensitive measure of airflow obstruction.
  • Early detection and treatment of pulmonary exacerbations in CF are crucial.

Purpose of the Study:

  • To investigate if using an increased lung clearance index (LCI) to trigger bronchoalveolar lavage (BAL) and antimicrobial treatment improves clinical outcomes in children with CF.
  • To assess the impact of LCI-guided intervention on lung function, nutritional status, and respiratory symptoms.

Main Methods:

  • A 2-year, randomized, controlled pilot study involving 29 children (5-18 years) with CF.
  • Quarterly assessments of LCI, z-scores for forced expired volume in 1s (zFEV1), and z-scores for body mass index (zBMI).
  • Intervention group received BAL and antimicrobial treatment if LCI increased by >1 unit from baseline; control group received standard care.

Main Results:

  • No significant difference in the rate of LCI decline between the intervention and control groups (0.21 units/year).
  • Secondary outcomes including zFEV1, zBMI, CFQ-R respiratory symptom scores, and CT-derived lung disease measures also showed no significant group differences.
  • The intervention group underwent more BAL procedures (median 6.0 vs 2.5).

Conclusions:

  • LCI-triggered BAL and antimicrobial treatment did not demonstrate clinical benefit in this cohort of closely monitored pediatric CF patients.
  • The study suggests that this LCI-guided strategy may not be an effective approach for managing CF exacerbations.
  • Further research with larger cohorts may be needed to confirm these findings.
Abstract

Keywords:
Bronchoalveolar lavage (BAL)Computed tomographyCystic fibrosisMultiple breath washoutPaediatric lung function testing

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