129Xe ventilation MRI to assess regional response to personalised airway clearance techniques in children with Primary ciliary dyskinesia
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All Authors
Schofield, Lynne
Biancardi, Alberto
Smith, Laurie
Marshall, Helen
Hughes, David
Capener, David
Bray, Jody
Fazal, Shahgufta
Zalewska, Anna
Norquay, Graham
LTHT Author
Schofield, Lynne
Robson, Evie
Robson, Evie
LTHT Department
Physiotherapy
Leeds Children's Hospital
Cystic Fibrosis Centre
Leeds Children's Hospital
Cystic Fibrosis Centre
Non Medic
Publication Date
2025-07-10
Item Type
Conference Abstract
Language
en
Subject
cystic fibrosis , primary ciliary dyskinesia , airway clearance technique , regional lung ventilation
Subject Headings
Abstract
Introduction
Children with Primary ciliary dyskinesia (PCD) use personalised airway clearance techniques (ACT), yet their effects on regional lung ventilation are unknown. 129Xe Ventilation MRI (129Xe-MRI) images lung ventilation distribution. 129Xe-MRI ventilation defect percentage (VDP) is an established whole lung metric but 129Xe-MRI can also provide regional lung ventilation information.
Aim
We aimed to assess the effects of a single personalised ACT regimen on regional ventilation using 129Xe-MRI.
Methods
Children with PCD were imaged with 129Xe-MRI pre-, post- and 4-hours post personalised ACT regimen. Regional ventilation was assessed by categorising each image voxel according to signal intensity. Ventilation was categorised as: high, normal, low, or ventilation defect (VDP). For treatment response we calculated; the proportion of pre-ACT defect that persisted (P-VD) and the proportion of lung volume changing to or from low ventilation or VDP (%change). Data presented as median (IQR) or mean [SD].
Results
26 children were assessed (14 male, age 7-17years, FEV1 z-score=-1.5 [1.6]). Most had visible defects at baseline pre-ACT VDP= 6.0 (12.8)%. Post-ACT more than 50% of baseline VDP had improved in most individuals (20/26 (76.9)%)), but some individuals had significant persistent defects; P-VD = 23.9 (36.2)%. Widespread ventilation changes were seen %change = 21.3 (15.4)%. Similar changes were seen 4-hours post-ACT: P-VD = 21.4 (36.9)%; %change = 20.8 (13.1)%.
Both the P-VD and %change assessed post-ACT correlated significantly with baseline VDP: (r=0.8, p<0.001 and r=0.9, p<0.001 respectively). Similar correlations were seen 4-hours post-ACT; (r=0.8, p<0.001 and r=0.9, p<0.001 respectively).
Conclusion
129Xe-MRI revealed widespread ventilation changes following a single ACT regimen. Children with more advanced disease have higher persistence of ventilation defects following ACT. ACTs are widely used in chronic lung diseases.129Xe-MRI can provide novel regional information on the effects of ACT regimens which may inform both future research and clinical practice.