Pediatric pulmonology · 2025
Airway Disease Progression on Chest Computed Tomography in Children With Primary Ciliary Dyskinesia.
СтатьяМногоцентровое исследованиеНаблюдательное исследованиеНазвание и аннотация: Перевод в очереди
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Kinghorn BreAnna, Mollica Federico, Caudri Daan, Davis Stephanie D, Dell Sharon, Ferkol Thomas W, Gardner Robert Andrew, Knowles Michael R, Milla Carlos, Pittman Jessica E, Rosenfeld Margaret, Sagel Scott D, Shapiro Adam J, Sullivan Erin, Sullivan Kelli M, Tiddens Harm A W M, Zariwala Maimoona A, Leigh Margaret W, Genetic Disorders of Mucociliary Clearance Consortium
Аннотация
RATIONALE: The longitudinal trajectory of airway disease on chest computed tomography (CT) and risk factors for progression in primary ciliary dyskinesia (PCD) is largely unknown.
METHODS: Longitudinal analysis of structural airway disease among children with PCD enrolled in a prospective, multicenter observational study, stratified by ultrastructural defect group. CTs were scored utilizing the Melbourne-Rotterdam Annotated Grid Morphometric Analysis for PCD, evaluating airway abnormalities in a hierarchical order: atelectasis, bronchiectasis (%BE), airway wall thickening, and mucus plugging/tree in bud opacities (%MP). Volume fraction of each component was expressed as % of total lung volume. %Disease (%DIS) is the sum of all components. Mixed effects regression analyses were utilized to describe the association between clinical predictors and CT scores adjusted for relevant covariates.
RESULTS: Chest CTs (N = 424) were obtained from 142 children over a mean (SD) of 4.9 (3.7) years. Mean (SD) age at first CT was 8.5 (4.6) years. Mean %DIS increased with age for the cohort (0.09% per year, 95% CI: 0.02-0.15, p < 0.01), driven by increased %BE (0.06 higher per year, 95% CI: 0.04-0.08, p = 0.0001) and increased odds of MP (1.14 higher odds per year, 95% CI: 1.08-1.21, p < 0.0001). Children with inner dynein arm and microtubular defects had increased mean %DIS and increased odds of MP compared to other ciliary abnormalities (1.73% higher, 95% CI: 0.71-2.74, p = 0.001; 3.21 higher odds per year, 95% CI: 1.73-5.95, p < 0.001). Annual increase in CT scores did not differ between defect groups.
CONCLUSIONS: Children with PCD experience progressive bronchiectasis and worsening mucus plugging with age.
METHODS: Longitudinal analysis of structural airway disease among children with PCD enrolled in a prospective, multicenter observational study, stratified by ultrastructural defect group. CTs were scored utilizing the Melbourne-Rotterdam Annotated Grid Morphometric Analysis for PCD, evaluating airway abnormalities in a hierarchical order: atelectasis, bronchiectasis (%BE), airway wall thickening, and mucus plugging/tree in bud opacities (%MP). Volume fraction of each component was expressed as % of total lung volume. %Disease (%DIS) is the sum of all components. Mixed effects regression analyses were utilized to describe the association between clinical predictors and CT scores adjusted for relevant covariates.
RESULTS: Chest CTs (N = 424) were obtained from 142 children over a mean (SD) of 4.9 (3.7) years. Mean (SD) age at first CT was 8.5 (4.6) years. Mean %DIS increased with age for the cohort (0.09% per year, 95% CI: 0.02-0.15, p < 0.01), driven by increased %BE (0.06 higher per year, 95% CI: 0.04-0.08, p = 0.0001) and increased odds of MP (1.14 higher odds per year, 95% CI: 1.08-1.21, p < 0.0001). Children with inner dynein arm and microtubular defects had increased mean %DIS and increased odds of MP compared to other ciliary abnormalities (1.73% higher, 95% CI: 0.71-2.74, p = 0.001; 3.21 higher odds per year, 95% CI: 1.73-5.95, p < 0.001). Annual increase in CT scores did not differ between defect groups.
CONCLUSIONS: Children with PCD experience progressive bronchiectasis and worsening mucus plugging with age.