Original articlesStructural airway abnormalities in infants and young children with cystic fibrosis☆
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.
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Supported by grants from the Radiological Society of North America and the Cystic Fibrosis Foundation.