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Changes in Airway Dimensions on Computed Tomography Scans of Children with Cystic Fibrosis

Departments of Pediatric Pulmonology and Epidemiology and Biostatistics, Erasmus MC/Sophia Children's Hospital and University Medical Center, Rotterdam, The Netherlands; James Hogg iCAPTURE Center for Cardiovascular and Pulmonary Research, St. Paul's Hospital and University of British Columbia; Department of Radiology, Vancouver General Hospital, Vancouver, British Columbia, Canada; Columbus Children's Hospital, Children's Radiological Institute, Columbus, Ohio; and Department of Respiratory Medicine, Shiga University of Medical Science, Otsu, Shiga, Japan

Correspondence and requests for reprints should be addressed to Harm A. Tiddens, M.D., Ph.D., Erasmus MC, Sophia Children's Hospital, Department of Pediatric Pulmonology, Dr Molewaterplein 60, 3015 GJ Rotterdam, the Netherlands. E-mail: h.tiddens{at}erasmusmc.nl

Rationale: In cystic fibrosis (CF), chronic bacterial infection and inflammation lead to progressive airway wall thickening and lumen dilatation. Objectives: To quantify airway wall thickening and lumen dilatation in children with CF over a 2-year interval. Methods: Children with CF (n = 23) who had two computed tomography (CT) scans (CT_cf1 and CT_cf2) combined with pulmonary function tests (PFTs), with a 2-year interval between measurements, were compared with control subjects (n = 21) who had one CT (CT_controls). On cross-sectional cut airway–artery pairs, airway wall area (WA), airway lumen area (LA) and perimeter, and arterial area (AA) were quantified. LA/AA (= marker of bronchiectasis), airway wall thickness (AWT), and WA/AA (= markers of wall thickness) were calculated. CT scans were scored using four different scoring systems. PFTs were expressed as percent predicted. Results: Airway WA-to-AA ratio was 1.45 (p < 0.001) and airway LA-to-AA ratio was 1.92 times higher (p < 0.001) in children with CF compared with age-matched control subjects. LA/AA and WA/AA remained unchanged from CT_cf1 to CT_cf2 and did not increase with age. AWT as a function of airway size increased from CT_cf1 to CT_cf2 by 2% (0.03 mm; p = 0.02). The change in AWT was inversely related to the change in forced expiratory flow between 25 and 75% of expiratory VC (p = 0.002). Conclusions: In CF, quantitative measurements of airways on CT scans show an increased ratio between airway LA and AA and progressive airway wall thickening. Scoring systems show progression of bronchiectasis but unchanged AWT. PFTs remained stable.

Key Words: airway wall • child • computed tomography • cystic fibrosis • quantitative evaluation

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