Longitudinal lung function and structural changes in children with primary ciliary dyskinesia
Autor: | Estelle Escudier, Aline Tamalet, Hubert Ducou Le Pointe, Malika Mahloul, Pierrick Cros, Sylvain Blanchon, Nicole Beydon, Annick Clement, Marie Lémery Magnin |
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Rok vydání: | 2012 |
Předmět: |
Pulmonary and Respiratory Medicine
Spirometry medicine.medical_specialty Pulmonary function testing 03 medical and health sciences FEV1/FVC ratio 0302 clinical medicine Internal medicine medicine 030212 general & internal medicine 10. No inequality Prospective cohort study Primary ciliary dyskinesia Lung Bronchiectasis medicine.diagnostic_test business.industry medicine.disease 3. Good health Surgery Peribronchial Thickening medicine.anatomical_structure 030228 respiratory system Pediatrics Perinatology and Child Health medicine.symptom business |
Zdroj: | Pediatric Pulmonology. 47:816-825 |
ISSN: | 8755-6863 |
Popis: | Background and Objectives 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. Materials and 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. Conclusion 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. Pediatr Pulmonol. 2012. 47:816–825. © 2012 Wiley Periodicals, Inc. |
Databáze: | OpenAIRE |
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