title: Longitudinal lung function and structural changes in children with primary ciliary dyskinesia.
abstract: OBJECTIVE
  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.
  
  
  METHODS
  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.
  
  
  RESULTS
  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.
  
  
  CONCLUSIONS
  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.
authors.affiliation: Assistance Publique-Hôpitaux de Paris, Hôpital Armand-Trousseau, Service de Radiologie Pédiatrique, Paris, France.
authors.forename: Marie Lémery
authors.initials: ML
authors.lastname: Magnin
authors.forename: Pierrick
authors.initials: P
authors.lastname: Cros
authors.forename: Nicole
authors.initials: N
authors.lastname: Beydon
authors.forename: Malika
authors.initials: M
authors.lastname: Mahloul
authors.forename: Aline
authors.initials: A
authors.lastname: Tamalet
authors.forename: Estelle
authors.initials: E
authors.lastname: Escudier
authors.forename: Annick
authors.initials: A
authors.lastname: Clément
authors.forename: Hubert Ducou
authors.initials: HD
authors.lastname: Le Pointe
authors.forename: Sylvain
authors.initials: S
authors.lastname: Blanchon
mesh_terms: D000293:Adolescent; D000328:Adult; D001784:Blood Gas Analysis; D002648:Child; D002675:Child, Preschool; D018450:Disease Progression; D005260:Female; D006801:Humans; D007223:Infant; D007619:Kartagener Syndrome / Q000000981:diagnostic imaging / Q000503:physiopathology*; D008137:Longitudinal Studies; D008168:Lung / Q000000981:diagnostic imaging / Q000503:physiopathology*; D008297:Male; D012129:Respiratory Function Tests; D012189:Retrospective Studies; D014057:Tomography, X-Ray Computed