Longitudinal lung function and structural changes in children with primary ciliary dyskinesia.

Details

Serval ID
serval:BIB_09DC93150171
Type
Article: article from journal or magazin.
Collection
Publications
Institution
Title
Longitudinal lung function and structural changes in children with primary ciliary dyskinesia.
Journal
Pediatric pulmonology
Author(s)
Magnin M.L., Cros P., Beydon N., Mahloul M., Tamalet A., Escudier E., Clément A., Le Pointe H.D., Blanchon S.
ISSN
1099-0496 (Electronic)
ISSN-L
1099-0496
Publication state
Published
Issued date
08/2012
Peer-reviewed
Oui
Volume
47
Number
8
Pages
816-825
Language
english
Notes
Publication types: Journal Article
Publication Status: ppublish
Abstract
Functional and structural lung evaluations are part of the follow-up of patients with primary ciliary dyskinesia (PCD). We aimed to evaluate transversal and longitudinal relationships between lung function test (LFT) and chest computed tomography (CT) in children with PCD, in stable clinical condition.
Data from children followed in the French National Center were retrospectively collected. Inclusion criteria were (i) definitive diagnosis of PCD, (ii) age less than 15 years at the beginning of follow-up, (iii) at least 8 years of follow-up, (iv) at least two couples of concurrent CT and LFT available in a phase of clinical stability of the lung disease without modification of the treatment regimen in the last 4 weeks. Twenty children (median age at entry 4.6 years, median follow-up 15.4 years) were included. Concurrent LFT (blood gas and spirometry) and CT (score) results were recorded.
LFT indices (PaO(2) (n = 210), FVC, FEV(1) , FEF(2575%) (n = 195)) significantly decreased with age, and the mean annual decrease (z-score (% predicted)) was -0.17 (-0.49%), -0.09 (-0.50%), -0.10 (-0.89%), and -0.07 (-1.73%), respectively. First CT (median age 8.7 years) revealed bronchiectasis (70%), mucous plugging (70%), peribronchial thickening (90%), parenchymal abnormalities (65%), and hyperinflation (50%). CT scores (n = 74) significantly increased with age, and was negatively correlated to PaO(2), FVC, FEV(1), and FEF(2575%) longitudinal changes.
In stable clinical condition, functional, and structural progressive impairments significantly correlated in children with PCD. Further prospective studies, including large populations of patients with various levels of disease severity, are needed to confirm whether lung function follow-up can be used to adjust CT frequency and help at minimizing the radiation burden in children with a good life expectancy.
Keywords
Adolescent, Adult, Blood Gas Analysis, Child, Child, Preschool, Disease Progression, Female, Humans, Infant, Kartagener Syndrome/diagnostic imaging, Kartagener Syndrome/physiopathology, Longitudinal Studies, Lung/diagnostic imaging, Lung/physiopathology, Male, Respiratory Function Tests, Retrospective Studies, Tomography, X-Ray Computed
Pubmed
Web of science
Create date
15/05/2020 16:27
Last modification date
16/05/2020 5:26
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