Original Article
High-resolution computed tomography in young patients with cystic fibrosis: Distribution of abnormalities and correlation with pulmonary function tests

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Abstract

Objective

To assess pulmonary abnormalities detected by high-resolution computed tomography (HRCT) in young children with cystic fibrosis (CF) and mild to moderate lung disease.

Study design

High-resolution computed tomography was performed in 60 children, 6 to 10 years old, with mild to moderate lung disease (forced expiratory volume in 1 minute [FEV1], 52–137; mean, 102; SD, 15% predicted). HRCTs were scored by using a system that evaluates each lobe for severity and extent of CF lung disease. Findings of CF lung disease were tabulated in all subjects and in a subgroup with normal pulmonary function tests. HRCT scores were correlated with FEV1, forced vital capacity (FVC), and forced expiratory flow between 25% and 75% of expired vital capacity (FEF25-75) in 57 patients.

Results

Bronchiectasis was present in 35% of subjects, mucous plugging in 15%, and air trapping in 63%. No abnormality was detected in 25%. In 37 subjects with FEV1, FVC, and FEF25-75 >85% predicted, bronchiectasis was present in 30%. In 17% of these subjects, bronchiectasis was seen in ≥4 lobes. Correlations between HRCT scores and FEV1 were significant and showed fair to moderate correlation (r = 0.36–0.46).

Conclusions

High-resolution computed tomography demonstrated a broad range of pulmonary abnormalities in young patients with CF with mild to moderate lung disease. In this study, abnormalities, including bronchiectasis, were common in young children with CF and normal 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

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  • Cited by (0)

    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.

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