Airway wall thickening and mucus plugging are important characteristics of cystic fibrosis (CF) lung disease in the first 5 years of life.The aim of this study is to investigate the association of lung disease in preschool children (age, 2-6) with bronchiectasis and other clinical outcome measures in the school age (age >7). Deidentified computed tomography-scans were annotated using Perth-Rotterdam annotated grid morphometric analysis for CF. Preschool %disease (a composite score of %airway wall thickening, %mucus plugging, and %bronchiectasis) and %MUPAT (a composite score of %airway wall thickening and %mucus plugging) were used as predictors for %bronchiectasis and several other school-age clinical outcomes. For statistical analysis, we used regression analysis, linear mixed-effects models and two-way mixed models. Sixty-one patients were included. %Disease increased significantly with age (P <.01). Preschool %disease and %MUPAT were significantly associated with school-age %bronchiectasis (P <.01 and P <.01, respectively). No significant association was found between preschool %disease and %MUPAT and school-age forced expiratory volume 1 (FEV1%) predicted and quality of life (P >.05). Cross-sectional, %disease in school-age was associated with a low FEV1% predicted and low quality of life (P =.01 and P =.007, respectively). %Disease can be considered an early marker of diffuse airways disease and is a risk factor for school-age bronchiectasis.

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Pediatric Pulmonology
Department of Biostatistics

Bouma, N.R. (Nynke R.), Janssens, H., Andrinopoulou, E.-R., & Tiddens, H. (2019). Airway disease on chest computed tomography of preschool children with cystic fibrosis is associated with school-age bronchiectasis. Pediatric Pulmonology. doi:10.1002/ppul.24498