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

J Cyst Fibros. 2020 Nov;19(6):934-941. doi: 10.1016/j.jcf.2020.06.010. Epub 2020 Jun 21.

Abstract

Hypothesis: Using increase in the lung clearance index (LCI) as a trigger for bronchoalveolar lavage (BAL) and associated antimicrobial treatment might benefit clinical outcomes in children with cystic fibrosis (CF).

Methods: A 2-year, longitudinal, interventional, randomized, controlled pilot study with quarterly visits in 5-18 years old children with CF. LCI and z-scores for the forced expired volume in 1 s (zFEV1) and body mass index (zBMI) were obtained at every visit, CF Questionnaire-revised (CFQ-R) yearly and BAL and chest computed tomography at first and last visit. Children in the intervention group had BAL performed if LCI increased >1 unit from a fixed baseline value established at first visit. If the presence of a pathogen was documented in the BAL fluid, treatment was initiated/altered accordingly.

Results: Twenty-nine children with CF were randomized to the control (n = 14) and intervention group (n = 15). The median (interquartile range) number of BAL procedures per child was 2.5 (2.0; 3.0) and 6.0 (4.0; 7.0) in the control and intervention group, respectively. There was no significant difference between groups in slope for the primary outcome LCI; difference was 0.21 (95% confidence interval: -0.45; 0.88) units/year. Likewise, there was no significant difference between groups in slope for the secondary outcomes zFEV1, zBMI, CFQ-R respiratory symptom score and the proportion of total disease and trapped air on chest computed tomography.

Conclusions: LCI-triggered BAL and associated antimicrobial treatment did not benefit clinical outcomes in a small cohort of closely monitored school-age children with CF.

Keywords: Bronchoalveolar lavage (BAL); Computed tomography; Cystic fibrosis; Multiple breath washout; Paediatric lung function testing.

Publication types

  • Randomized Controlled Trial
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adolescent
  • Anti-Bacterial Agents / therapeutic use*
  • Bacterial Load
  • Bronchoalveolar Lavage Fluid / microbiology*
  • Child
  • Child, Preschool
  • Cystic Fibrosis / drug therapy*
  • Cystic Fibrosis / microbiology*
  • Cystic Fibrosis / physiopathology*
  • Disease Progression
  • Female
  • Humans
  • Longitudinal Studies
  • Male
  • Pilot Projects
  • Respiratory Function Tests*

Substances

  • Anti-Bacterial Agents