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Pulmonary function in hospitalized infants and toddlers with cystic fibrosis
R G Clayton1, C E Diaz, N S Bashir
1Department of Pediatrics, Temple University School of Medicine, St. Christopher's Hospital for Children, Philadelphia, Pennsylvania 19134, USA.
Insights
Hospitalization for cystic fibrosis (CF) pulmonary exacerbations significantly improves lung function in infants and toddlers. Measurements like maximal flow at functional residual capacity (VmaxFRC) showed the greatest gains, indicating better outcomes for those with poorer initial lung function.
Area of Science:
- Pediatric Pulmonology
- Respiratory Medicine
- Cystic Fibrosis Research
Background:
- Hospitalization for pulmonary exacerbations in older children with cystic fibrosis (CF) is associated with documented improvements in lung function.
- Limited data exist on lung function changes in infants and toddlers with CF during hospitalization for pulmonary exacerbations.
Purpose of the Study:
- To determine if lung function improves in infants and toddlers hospitalized with CF pulmonary exacerbations.
- To compare lung function changes measured by forced expiratory flow and tidal breathing techniques.
Main Methods:
- Seventeen infants and toddlers with CF were assessed at admission and discharge.
- Maximal flow at functional residual capacity (VmaxFRC) was measured using the rapid thoracic compression technique.
- Lung conductance (GL) and lung compliance (CL) were measured using esophageal balloons to assess tidal mechanics.
Main Results:
- Significant improvements in lung function were observed during hospitalization.
- Maximal flow at functional residual capacity (VmaxFRC) increased from 38.5% to 59.8% predicted (p < 0.005).
- Lung conductance (GL) and lung compliance (CL) also improved significantly (p < 0.02 and p < 0.03, respectively).
- Greater improvement was seen in patients with poorer baseline pulmonary function.
Conclusions:
- Hospitalization leads to improved lung function in infants and toddlers with CF.
- Maximal flow at functional residual capacity (VmaxFRC) may better reflect the primary lung disease pathophysiology in this age group compared to lung conductance (GL).
- Discrepancies between VmaxFRC and GL findings may arise from measuring different physiological properties.
Unlabelled:
In older children with cystic fibrosis (CF), well-documented improvements in lung function occur during hospitalization for treatment of pulmonary exacerbations.
Objectives:
(1) To test the hypothesis that improvement in lung function occurs in infants and toddlers hospitalized because of CF pulmonary exacerbations. (2) To compare changes in lung function measured during forced expiratory flow and tidal breathing.
Study Design:
Seventeen infants and toddlers with CF were evaluated at the beginning and end of hospitalization by the rapid thoracic compression technique to yield maximal flow at forced residual capacity. Tidal mechanics were measured by the esophageal balloon technique to yield lung conductance and compliance.
Results:
Lung function improved during the course of hospitalization. The greatest change was observed in measurements of maximal flow at functional residual capacity (.VmaxFRC), increasing from 38.5% +/- 6% predicted (mean +/- SEM) to 59.8% +/- 6% at the end (p < 0.005). Lung conductance (GL) increased from 60% +/- 6% to 78% +/- 8% (p < 0.02); lung compliance (CL) increased from 66% +/- 5% to 75% +/- 5% (p < 0.03). The degree of improvement of .VmaxFRC, GL, and CL was related to baseline measurements; those with poorer pulmonary function at baseline had the greatest degree of improvement during hospitalization.
Conclusion:
Assessments of airflow obstruction from measurements of .VmaxFRC and GL do not necessarily demonstrate similar findings in a given infant with CF, perhaps because these two techniques measure different physiologic properties. Changes in .VmaxFRC may best reflect the predominant pathophysiology of lung disease in infants and toddlers with CF.
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