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.

Janus kinase inhibitors display broad anti-itch properties: A possible link through the TRPV1 receptor

“…In this study we observed that the JAK inhibitors oclacitinib and tofacitinib exhibit broad anti-itch properties that appear linked to their inhibition of transient receptor potential cation channel subfamily V member 1 (TRPV1), a receptor known to play an important role in the transmission of both pain and itch. 4 To illustrate the involvement of TRPV1 in the anti-itch properties of JAK inhibitors, we used 3 different approaches: (1) in vitro calcium imaging in DRG neurons; (2) in vivo methods with a mouse model of itch and one of capsaicin-induced pain; and (3) TRPV1-transfected AD293 cells.…”

Small airways diseases, excluding asthma and COPD: an overview

“…Low diffusing capacity of the lung for carbon monoxide (DLCO) was associated with bronchial wall thickening and linear opacities. In another study of a cohort of 51 children with CVID, the authors reported correlations between air trapping on CT scans and distal airway obstruction, as measured by FEF25-75% [168].…”

The Role of Small Airways in Childhood Asthma

“…67 While no pediatric asthma studies are available, in other pediatric obstructive disease processes, HRCT correlates of small-airway disease are seen. 68,69 Partly due to the ionizing radiation hazards of repeated HRCT scanning, there is emerging interest in the use of hyperpolarized gases as contrast agents for MRI. When there is a ventilation defect, the helium-3 or xenon-129 does not fill the airspaces, leading to a dark area on an otherwise high signal throughout the lung.…”