Elsevier

The Journal of Pediatrics

Volume 144, Issue 2, February 2004, Pages 154-161
The Journal of Pediatrics

Original articles
Structural airway abnormalities in infants and young children with cystic fibrosis

https://doi.org/10.1016/j.jpeds.2003.09.026Get rights and content

Abstract

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.

Section snippets

Subjects

High-resolution computed tomographic imaging of the lungs was performed in infants and young children with CF, age 0 to 5 years, who were considered by their attending physicians to be clinically well at the time of the study. The diagnosis of CF was confirmed by either duplicate quantitative pilocarpine iontophoresis sweat testing or genotyping. Children with CF were studied either when referred to the infant pulmonary function laboratory for their routine 6-month evaluation or when they had

Results

Infants and young children with CF (18 boys, 16 girls; mean age, 2.4±1.4 years; range, 10 weeks to 5.5 years) and 20 normal control infants and young children (12 boys, 8 girls; mean age, 1.8±1.4 years; range, 1 week to 5 years) were studied. Thirty-five of 39 eligible children age 0 to 5 years were recruited over a 2-year period. One child awoke in the scanner before imaging, and the study was not completed. The mean ages of children with CF and normal control children did not differ

Discussion

This study demonstrates that the airways of minimally symptomatic infants and young children with CF have thicker walls and are more dilated than those of normal control infants. Accompanying pulmonary vessels were, on average, smaller in patients with CF. Ratios of both AWT and ALD to accompanying vessel diameter were significantly increased in patients with CF. The mean degree to which airways were dilated increased with increasing patient age. These structural changes were seen in both

Acknowledgements

We thank Robert Flucke, Joseph Good, and Dave Filbrun for their invaluable assistance in performing the studies and processing the data, and Dr John Hayes and Dr Stacy Hoshaw-Woodard for their guidance in the analysis of the data.

References (26)

  • C.W Bedrossian et al.

    The lung in cystic fibrosis: a quantitative study including prevalence of pathologic findings among different age groups

    Hum Pathol

    (1976)
  • A Cantin

    Cystic fibrosis lung inflammation: early, sustained, and severe

    Am J Respir Crit Care Med

    (1995)
  • K Balough et al.

    The relationship between infection and inflammation in the early stages of lung disease from cystic fibrosis

    Pediatr Pulmonol

    (1995)
  • T.Z Khan et al.

    Early pulmonary inflammation in infants with cystic fibrosis

    Am J Respir Crit Care Med

    (1995)
  • D.S Armstrong et al.

    Lower airway inflammation in infants and young children with cystic fibrosis

    Am J Respir Crit Care Med

    (1997)
  • J.L Burns et al.

    Longitudinal assessment of Pseudomonas aeruginosa in young children with cystic fibrosis

    J Infect Dis

    (2001)
  • J.R Esterly et al.

    Observations in cystic fibrosis of the pancreas, 3: pulmonary lesions

    Johns Hopkins Med J

    (1968)
  • F.R Long et al.

    Lungs in infants and young children: improved thin-section CT with a noninvasive controlled-ventilation technique—initial experience

    Radiology

    (1999)
  • F Long et al.

    Technique and clinical applications of full inflation and end-exhalation controlled-ventilation chest CT in infants and young children

    Pediatr Radiol

    (2001)
  • G.G King et al.

    Evaluation of airways in obstructive pulmonary disease using high-resolution computed tomography

    Am J Respir Crit Care Med

    (1999)
  • A.E McNamara et al.

    Airway narrowing in excised canine lungs measured by high-resolution computed tomography

    J Appl Physiol

    (1992)
  • M Okazawa et al.

    Human airway narrowing measured using high resolution computed tomography

    Am J Respir Crit Care Med

    (1996)
  • R Castile et al.

    Adult-type pulmonary function tests in infants without respiratory disease

    Pediatr Pulmonol

    (2000)
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