Lung function deterioration in school children with cystic fibrosis
Katarzyna Walicka-Serzysko1,2, Magdalena Postek1,2, Justyna Milczewska1,2
1Cystic Fibrosis Department, Institute of Mother and Child, Warsaw, Poland.
Insights
Early lung disease in cystic fibrosis (CF) children shows increased ventilation inhomogeneity, airway obstruction, and trapped gas with age. Multiple-breath nitrogen washout (MBNW) can detect these lung function changes in children unable to perform spirometry.
Area of Science:
- Pediatric Pulmonology
- Respiratory Medicine
- Cystic Fibrosis Research
Background:
- Lung disease in cystic fibrosis (CF) manifests early, yet detecting pulmonary dysfunction in children is challenging.
- Current methods for assessing early lung abnormalities in pediatric CF patients have limitations.
Purpose of the Study:
- To evaluate the early progression of lung function in children with CF.
- To analyze the age-related changes in pulmonary hyperinflation, ventilation inhomogeneity (VI), trapped gas, and airway obstruction.
Main Methods:
- One hundred CF children (aged 7-18) were divided into two age groups (7-12 and 13-18 years).
- Multiple-breath nitrogen washout (MBNW) and plethysmography were used to measure lung clearance index (LCI), functional residual capacity (FRC), trapped gas (VT), and airway resistance (Reff, sReff).
Main Results:
- A positive correlation was found between age and FRC (plethysmography and MBNW) and LCI.
- Ventilation inhomogeneity (VI) was higher in older children (11.67 vs. 9.79).
- Pulmonary hyperinflation, airway obstruction, and trapped gas were prevalent in a significant percentage of children, increasing with age.
Conclusions:
- Lung function in CF children gradually declines with increasing VI, airway obstruction, hyperinflation, and trapped gas.
- MBNW offers a viable alternative for measuring LCI and FRC (FRC MBNW) to assess lung function and hyperinflation in children unable to perform spirometry or plethysmography.
Introduction:
Lung disease in cystic fibrosis (CF) begins early in life but the capabilities for detecting abnormalities of pulmonary dysfunction in children remain limited.
Objective:
The study aimed to evaluate the early progression of lung function by the analysis of pulmonary hyperinflation, ventilation inhomogeneity (VI), trapped gas and airway obstruction with age.
Methods:
One hundred CF children aged 7 to 18, divided into two groups aged 7 to 12 (n = 40) and 13 to 18 (n = 60), were enrolled. Patients performed multiple-breath nitrogen washout (MBNW) tests and plethysmography for measurements of lung clearance index (LCI), functional residual capacity (FRCpleth , FRCMBNW ), volume of trapped gas (VT ), total resistance, and effective and specific effective airway resistance (Reff , sReff ).
Results:
We obtained a positive correlation of FRCpleth , FRCMBNW , and LCI with age. A linear correlation between FRCMBNW and FRCpleth (P < .0001) was observed. VI was higher in the group of older patients (9.79 in the group aged 7-12 and 11.67 in the group aged 13-18). An increased effective specific airway resistance >2 (z-score) was present in 58% of all subjects (50% and 63.3%, respectively). Pulmonary hyperinflation (FRCpleth >2 z-score) was observed in 33% of all patients: 25% and 36.6%, respectively. Trapped gas (VT > 2 z-score) was present in 18% of all children: 30% and 10%, respectively.
Conclusion:
A gradual decline in lung function is associated with an increase in VI, airway obstruction, pulmonary hyperinflation and development of trapped gas. In children who cannot perform either spirometry or plethysmography, MBNW can deliver a measurement of LCI connecting with VI as well as FRCMBNW to indicate indirectly the increase of hyperinflation.
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