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.
Abstract

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