Lung Clearance Index in Children with Cystic Fibrosis during Pulmonary Exacerbation
Katarzyna Walicka-Serzysko1,2, Magdalena Postek1,2, Justyna Milczewska1,2
1Cystic Fibrosis Department, Institute of Mother and Child, 01-211 Warsaw, Poland.
Insights
Pulmonary exacerbation (PEx) in cystic fibrosis (CF) significantly impacts lung function. The lung clearance index (LCI) detects impairment more effectively than FEV1 in children with CF during PEx.
Area of Science:
- Pediatric Pulmonology
- Respiratory Medicine
- Genetic Disorders
Background:
- Pulmonary exacerbation (PEx) is a critical factor influencing quality of life and survival in cystic fibrosis (CF) patients.
- Assessing lung function changes during PEx is vital for effective management.
Purpose of the Study:
- To evaluate changes in pulmonary function parameters, specifically the lung clearance index (LCI) and FEV1, in children with CF during PEx.
- To compare the sensitivity of LCI and FEV1 in detecting functional impairment during PEx.
Main Methods:
- A cohort of 40 children with CF (aged 6-17) was studied.
- Spirometry and multiple breath nitrogen washout (MBNW) tests were conducted during stable periods and after PEx treatment.
- Lung function parameters including LCI and FEV1 were analyzed before and after antibiotic therapy for PEx.
Main Results:
- During PEx, 40% of patients showed an LCI increase of 65% and a ≥10% decrease in FEV1.
- The absolute change in LCI was 1.05 units (11.48% relative change) and FEV1 decreased by -9.22% relative to baseline.
- Post-treatment, FEV1 improved by 11.05% and LCI decreased by 9.42% on average.
Conclusions:
- The lung clearance index (LCI) demonstrates a greater capacity to identify functional impairment in school-aged children with CF during pulmonary exacerbations compared to FEV1.
- LCI changes provide a more sensitive measure of lung disease activity in CF patients experiencing PEx.
Abstract:
(1) Background: Pulmonary exacerbation (PEx) is one of the main factors affecting the quality of life and life expectancy in patients with cystic fibrosis (CF). Our study aimed to evaluate the change in selected pulmonary function parameters, including lung clearance index (LCI), in patients with CF diagnosed with PEx. (2) Methods: We enrolled 40 children with CF aged 6-17. They performed spirometry and multiple breath nitrogen washout (MBNW) tests during a stable condition period at the beginning and the end of intravenous antibiotic treatment. (3) Results: LCI increased by 65% and FEV1 decreased by ≥10% in 40% of patients with CF during PEx. An absolute change in LCI between a stable condition period and PEx was 1.05 (±1.92) units, which corresponds to a relative change of 11.48% (±18.61) of the baseline. The relative decrease in FEV1 was -9.22% (±12.00) and the z-score was -0.67 (±1.13). After the PEx treatment, FEV1 increased by 11.05% (±9.04) on average, whereas LCI decreased by 1.21 ± 1.59 units on average, which represented 9.42% ± 11.40 compared to the value at the beginning of PEx. (4) Conclusions: The change in LCI captures a higher proportion of events with functional impairment than FEV1 in school-age children with CF.
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