Longitudinal lung function and structural changes in children with primary ciliary dyskinesia
Pediatric Pulmonology,

Magnin ML et al. – In stable clinical condition, functional, and structural progressive impairments significantly correlated in children with primary ciliary dyskinesia (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.

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 (PaO2 (n=210), FVC, FEV1, FEF2575% (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 PaO2, FVC, FEV1, and FEF2575% longitudinal changes.

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