title: High-resolution computed tomography and pulmonary function in children with common variable immunodeficiency.
abstract: High-resolution computed tomography (HRCT) may be useful to monitor lung disease in children with common variable immunodeficiency disorder (CVID). We evaluated interobserver agreement and correlation with pulmonary function tests (PFTs) for automated quantification and visual scoring of air trapping and airway wall thickening on HRCT in paediatric CVID patients. In a cohort of 51 children with CVID, HRCT was analysed visually and automated for presence of air trapping and airway wall thickening. PFTs were expressed as % predicted. Disease duration, physician-diagnosed pneumonias and antibiotic prophylaxis were recorded. Interobserver agreement for automated airway wall thickening was good with an intra-class correlation coefficient of 0.88, compared with 0.51 for visual scoring. Presence of air trapping on HRCT correlated significantly with PFTs and disease duration, but was not associated with previous pneumonias. Airway wall thickening did not correlate significantly with PFTs or disease duration and was not associated with previous pneumonias or prophylactic antibiotic use. In children with CVID disorders, HRCT air trapping measurements are significantly correlated with PFTs and disease duration. Quantitative air trapping is a feasible and promising technique for small airway disease quantification that may be applied to monitor (silent) disease progression in CVID.
authors.affiliation: Dept of Radiology, University Medical Center Utrecht, Utrecht, The Netherlands.
authors.forename: L
authors.initials: L
authors.lastname: van Zeggeren
authors.forename: A A J M
authors.initials: AA
authors.lastname: van de Ven
authors.forename: S W J
authors.initials: SW
authors.lastname: Terheggen-Lagro
authors.forename: O M
authors.initials: OM
authors.lastname: Mets
authors.forename: F J
authors.initials: FJ
authors.lastname: Beek
authors.forename: J M
authors.initials: JM
authors.lastname: van Montfrans
authors.forename: P A
authors.initials: PA
authors.lastname: de Jong
mesh_terms: D000293:Adolescent; D000900:Anti-Bacterial Agents / Q000627:therapeutic use; D002648:Child; D015331:Cohort Studies; D017074:Common Variable Immunodeficiency / Q000150:complications / Q000000981:diagnostic imaging / Q000503:physiopathology*; D018450:Disease Progression; D005260:Female; D006801:Humans; D008171:Lung Diseases / Q000000981:diagnostic imaging* / Q000188:drug therapy / Q000209:etiology / Q000503:physiopathology*; D008297:Male; D015588:Observer Variation; D012129:Respiratory Function Tests; D014057:Tomography, X-Ray Computed / Q000379:methods
chemical_list: D000900:Anti-Bacterial Agents