Original ArticleHigh-resolution computed tomography in young patients with cystic fibrosis: Distribution of abnormalities and correlation with pulmonary function tests☆
Section snippets
Materials and methods
Initial HRCTs and Pulmonary function tests (PFTs) from 60 subjects participating in the Pulmozyme Early Intervention Trial7 at 15 centers were reviewed. The HRCT arm of the study and the overall trial were approved by the Institutional Review Board of each center. All Institutional Review Board applications submitted for this study were approved. The CF centers and the principal investigator at each site are listed in the Appendix. Eligibility criteria included a diagnosis of CF, routine
Findings of cystic fibrosis lung disease
Agreement between readers for the presence or absence of findings of CF lung disease was 83% for bronchiectasis, 93% for mucous plugging, and 82% for air trapping.
Examples of the appearance of different features of CF lung disease are shown in Fig 2. The distribution of findings of CF lung disease is shown in Fig 1 (available online at www.us.elsevierhealth.com/jpeds).
Air trapping was the most common abnormality, occurring in 38 subjects (63%) and in 163 of 360 lobes (45%). When present, air
Discussion
This is the largest reported study of computed tomographic findings in children with CF.4., 11., 12. This study is unique in that the subjects were all 6 to 10 years old with screening FVC values in the normal range (≥85% predicted). This study group allowed us to evaluate the range and severity of pulmonary disease in young patients with mild (FEV1 >70% predicted) and moderate (FEV1 40%–69% predicted) lung disease. Although 65% of our subjects had normal PFTs, we include these subjects in the
References (24)
- et al.
High resolution computed tomography in adult cystic fibrosis patients with mild lung disease
Clin Radiol
(1991) - et al.
Outcome measures for clinical trials in cystic fibrosis: summary of a Cystic Fibrosis Foundation consensus conference
J Pediatr
(1994) - et al.
A two-year randomized, placebo-controlled trial of dornase alfa in young patients with cystic fibrosis with mild lung function abnormalities
J Pediatr
(2001) - et al.
High resolution CT in children with cystic fibrosis: correlation with pulmonary functions and radiographic scores
Eur J Radiol
(2001) - et al.
An automated approach to quantitative air trapping measurements in mild cystic fibrosis
Chest
(2003) - et al.
High resolution computed tomography of the chest in infants with cystic fibrosis
Pediatr Pulmonol
(1999) - et al.
Cystic fibrosis: scoring system with thin-section CT
Radiology
(1991) - et al.
Ultrafast computerized tomography of the chest in cystic fibrosis: a new scoring system
Pediatr Pulmonol
(1991) - et al.
Cystic fibrosis in children: HRCT findings and distribution of disease
J Thorac Imaging
(1996) - et al.
High-resolution computed tomography of the chest in children with cystic fibrosis: support for use as an outcome surrogate
Pediatr Radiol
(1999)
ATS statement—snowbird workshop on standardization of spirometry
Am Rev Respir Dis
Pulmonary function between 6 and 18 years of age
Pediatr Pulmonol
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Supplementary data associated with this article can be found at doi:10.1016/j.jpeds.2004.02.038
Supported by Cystic Fibrosis Foundation Grant BRO97.