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Spirometry in 3- to 6-year-old children with cystic fibrosis
Paulo J C Marostica1, Andrea D Weist, Howard Eigen
1Department of Pediatric Pulmonology and Critical Care, James Whitcomb Riley Hospital for Children, Indianapolis, Indiana 46202-5225, USA.
Insights
Spirometry effectively assesses lung function in preschool children with cystic fibrosis (CF), showing decreased pulmonary function but potential for early detection. This method allows for tracking CF progression from infancy through adulthood.
Area of Science:
- Pediatric Pulmonology
- Respiratory Medicine
- Genetic Diseases
Background:
- Spirometry is standard for older CF patients, but data for preschoolers is limited.
- Previous research established normative spirometry data for 3- to 6-year-old children.
Purpose of the Study:
- To assess pulmonary function in clinically stable preschool-aged children with cystic fibrosis (CF).
- To evaluate the feasibility and utility of spirometry in this age group for early CF detection and monitoring.
Main Methods:
- Thirty-eight preschool children with CF were enrolled; 33 completed technically acceptable spirometry maneuvers.
- Pulmonary function parameters (FVC, FEV(1), FEV(1)/FVC, FEF(25-75)) were analyzed as z scores.
- Correlations with radiological scores (Brasfield) and genetic mutations (DeltaF508) were examined.
Main Results:
- Preschoolers with CF demonstrated significantly decreased spirometry z scores compared to predicted values.
- Lower lung function correlated with higher Brasfield radiological scores.
- Homozygous DeltaF508 mutation carriers showed significantly lower FVC and FEV(1) z scores than heterozygous carriers.
Conclusions:
- Spirometry is a viable tool for assessing lung function in preschool children with CF.
- Early spirometry may enable longitudinal tracking of CF lung disease from infancy.
- Findings support the use of spirometry for early intervention and management in pediatric CF patients.
Abstract:
Spirometry is routinely used to assess pulmonary function of older children and adults with cystic fibrosis (CF); however, few data exist concerning the preschool age group. We have reported normative spirometric data for 3- to 6-year-old children. The current study was designed to assess a similarly aged group of clinically stable patients with CF. Thirty-three of 38 children with CF were able to perform 2 or 3 technically acceptable maneuvers. These patients had significantly decreased FVC, FEV(1), FEV(1)/FVC, and FEF(25-75) when expressed as z scores (number of SD from predicted): -0.75 +/- 1.63, -1.23 +/- 1.97, -0.87 +/- 1.33, and -0.74 +/- 1.63, respectively. There were significant positive correlations of the Brasfield radiological score with FVC and FEV(1) z scores (r(2) = 0.26, p < 0.01 and r(2) = 0.24, p < 0.01). In addition, homozygous patients for the DeltaF508 mutation had lower z scores for FVC (-1.21 versus 0.47, p < 0.01) and FEV(1) (-1.38 versus 0.21, p < 0.05) than heterozygous patients. Of the 14 patients who had full flow-volume spirometric measurements during infancy, 10 had FEF(25-75) z scores greater than -2 at both evaluations. Our findings suggest that spirometry can successfully be used to assess lung function in preschool children with CF and has the potential for longitudinal assessment from infancy through adulthood.