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

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HAL Id: inserm-00752965

https://www.hal.inserm.fr/inserm-00752965

Submitted on 16 Nov 2012

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Longitudinal lung function and structural changes in

children with primary ciliary dyskinesia

Sylvain Blanchon, Marie Magnin, Pierrick Cros, Estelle Escudier, Malika

Mahloul, Aline Tamalet, Annick Clément, Hubert Ducou Le Pointe, Nicole

Beydon

To cite this version:

Sylvain Blanchon, Marie Magnin, Pierrick Cros, Estelle Escudier, Malika Mahloul, et al.. Longitudinal

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

Cilia in Development and Disease Scientific Conference, pp.P3. �inserm-00752965�

(2)

P O S T E R P R E S E N T A T I O N

Open Access

Longitudinal lung function and structural

changes in children with primary ciliary

dyskinesia

S Blanchon

1*

, M Lémery Magnin

2

, P Cros

1

, E Escudier

3

, M Mahloul

4

, A Tamalet

1

, A Clement

1

, H Ducou Le Pointe

2

,

N Beydon

5

From First International Cilia in Development and Disease Scientific Conference (2012)

London, UK. 16-18 May 2012

Background

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 tests (LFT) and chest computed tomography (CT) in children with PCD, in stable clinical condition.

Methods

Data from children followed in the French National Cen-ter were retrospectively collected. Inclusion criCen-teria were (i) definitive diagnosis of PCD, (ii) age less than 15 years at the beginning of up, (iii) at least 8 years of follow-up and (iv) at least 2 cofollow-uples of concurrent CT and LFT performed in stable clinical condition. Twenty children (median age 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%), respec-tively. First CT (median age 8.7 years) revealed bronchiec-tasis (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 pro-gressive impairments significantly correlated in children with PCD. Further prospective studies, including large populations with various levels of disease severity, are needed to confirm whether LFT follow-up can be used to adjust CT frequency and help at minimizing the radiation burden in children with a good life expectancy.

Author details

1Department of Pediatric Pulmonology, Hôpital Trousseau, France. 2

Department of Pediatric Radiology, Hôpital Trousseau, France.3Department of Medical Genetics and Embryology, Hôpital Trousseau, France.4INSERM, Research

Unit 707, Hôpital Saint Antoine, France.5Lung Physiology, Hôpital Trousseau, France.

Published: 16 November 2012

doi:10.1186/2046-2530-1-S1-P3

Cite this article as: Blanchon et al.: Longitudinal lung function and structural changes in children with primary ciliary dyskinesia.Cilia 2012 1(Suppl 1):P3.

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Submit your manuscript at www.biomedcentral.com/submit * Correspondence: sylvainblanchon@hotmail.com

1Department of Pediatric Pulmonology, Hôpital Trousseau, France

Full list of author information is available at the end of the article Blanchonet al. Cilia 2012, 1(Suppl 1):P3

http://www.ciliajournal.com/content/1/S1/P3

© 2012 Blanchon et al; licensee BioMed Central Ltd. This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

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