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.
Pediatric Pulmonology
|August 8, 2020
Summary
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.
Keywords:
cystic fibrosisfunctional residual capacitylung clearance indexpulmonary function testingventilation inhomogeneityMore Related Videos
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