Structural airway abnormalities in infants and young children with cystic fibrosis

J Pediatr. 2004 Feb;144(2):154-61. doi: 10.1016/j.jpeds.2003.09.026.


Objectives: To determine whether the airway structure of infants and young children with cystic fibrosis (CF) differs from that of normal children by using high-resolution computed tomography (HRCT) imaging. Study design Full-inflation, controlled ventilation HRCT images of the lungs were obtained at four anatomic levels in 34 infants with CF (age, 2.4+/-1.4 years) and 20 control infants (age, 1.8+/-1.4 years). Short axis diameters of all clearly identifiable, round airway/vessel pairs were measured to obtain airway wall thickness (AWT), airway lumen diameter (ALD), and vessel diameter (VD).

Results: In infants with CF, mean AWT (+/-SD) was 0.58+/-0.13 mm, ALD was 1.31+/-0.56 mm, and VD was 1.62+/-0.58 mm. In control infants, mean AWT was 0.49+/-0.13 mm, ALD was 1.07+/-0.42 mm, and VD was 1.86+/-0.64 mm. Mean AWT and ALD were greater in children with CF than in normal subjects (P<.001). ALD:VD ratios increased with age in patients with CF compared with control subjects (P=.026).

Conclusions: The airways of infants and young children with CF have thicker walls and are more dilated than those of normal infants.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Analysis of Variance
  • Bronchi / pathology*
  • Bronchiectasis / diagnostic imaging*
  • Case-Control Studies
  • Child, Preschool
  • Cystic Fibrosis / diagnostic imaging
  • Cystic Fibrosis / pathology*
  • Dilatation, Pathologic / diagnostic imaging
  • Female
  • Humans
  • Infant
  • Male
  • Observer Variation
  • Positive-Pressure Respiration / methods
  • Pulmonary Artery / diagnostic imaging
  • Pulmonary Artery / pathology*
  • Pulmonary Veins / diagnostic imaging
  • Pulmonary Veins / pathology
  • Tomography, X-Ray Computed / methods