Automatic mucus plugging analysis on chest computed tomography in children with primary ciliary dyskinesia

<bold>RATIONALE:</bold> Primary ciliary dyskinesia (PCD) is characterized by impaired mucociliary clearance and progressive structural airway disease. Children with PCD and inner dynein arm and microtubular disorganization defects have worse structural airway disease compared to other ciliary defects. We utilized a novel automated method to quantify number and volume of mucus plugs (MPs) on chest CT in children with PCD. <bold>Methods:</bold> Cross-sectional analysis of chest CTs of children with PCD enrolled in a multicenter Genetic Disorders of Mucociliary Clearance Consortium observational study. CTs were analysed (LungQ-MP, Thirona, Netherlands) which automatically segments the bronchial tree, and measures the number and volume of MPs. <bold>Results:</bold> Mean(SD) <bold>Results:</bold> CTs were available from 141 children; 75 CTs met LungQ requirements (slice thickness ≤1.5 mm). A total of 433 MPs were detected (5.8(10.3) per CT), and volume of 0.3ml(0.7) per CT. Number MPs increased with age group: < 12 years: 2.8(5.1), 12-17 years: 5.0(6.7), and > 17 years: 12.8(17.9). <bold>Conclusions:</bold> The fully-automated LungQ-MP algorithm successfully detected MPs on chest CT. Substantial heterogeneity in the number of MPs was observed. We plan to evaluate the association between clinical predictors (including ciliary defect class), airway dimensions, and MPs.