Prevalence and course of disease after lung resection in primary ciliary dyskinesia: a cohort & nested case-control study.
Détails
Etat: Public
Version: Final published version
Licence: CC BY 4.0
ID Serval
serval:BIB_7F7D97A519C8
Type
Article: article d'un périodique ou d'un magazine.
Collection
Publications
Institution
Titre
Prevalence and course of disease after lung resection in primary ciliary dyskinesia: a cohort & nested case-control study.
Périodique
Respiratory research
Collaborateur⸱rice⸱s
Israeli PCD Consortium, Italian PCD Consortium, Swiss PCD Group, French Reference Centre for Rare Lung Diseases, PCD Italian Consortium, French Reference Centre for Rare Lung Diseases
ISSN
1465-993X (Electronic)
ISSN-L
1465-9921
Statut éditorial
Publié
Date de publication
18/09/2019
Peer-reviewed
Oui
Volume
20
Numéro
1
Pages
212
Langue
anglais
Notes
Publication types: Journal Article ; Multicenter Study
Publication Status: epublish
Publication Status: epublish
Résumé
Lung resection is a controversial and understudied therapeutic modality in Primary Ciliary Dyskinesia (PCD). We assessed the prevalence of lung resection in PCD across countries and compared disease course in lobectomised and non-lobectomised patients.
In the international iPCD cohort, we identified lobectomised and non-lobectomised age and sex-matched PCD patients and compared their characteristics, lung function and BMI cross-sectionally and longitudinally.
Among 2896 patients in the iPCD cohort, 163 from 20 centers (15 countries) underwent lung resection (5.6%). Among adult patients, prevalence of lung resection was 8.9%, demonstrating wide variation among countries. Compared to the rest of the iPCD cohort, lobectomised patients were more often females, older at diagnosis, and more often had situs solitus. In about half of the cases (45.6%) lung resection was performed before presentation to specialized PCD centers for diagnostic work-up. Compared to controls (n = 197), lobectomised patients had lower FVC z-scores (- 2.41 vs - 1.35, p = 0.0001) and FEV1 z-scores (- 2.79 vs - 1.99, p = 0.003) at their first post-lung resection assessment. After surgery, lung function continued to decline at a faster rate in lobectomised patients compared to controls (FVC z-score slope: - 0.037/year Vs - 0.009/year, p = 0.047 and FEV1 z-score slope: - 0.052/year Vs - 0.033/year, p = 0.235), although difference did not reach statistical significance for FEV1. Within cases, females and patients with multiple lobe resections had lower lung function.
Prevalence of lung resection in PCD varies widely between countries, is often performed before PCD diagnosis and overall is more frequent in patients with delayed diagnosis. After lung resection, compared to controls most lobectomised patients have poorer and continuing decline of lung function despite lung resection. Further studies benefiting from prospective data collection are needed to confirm these findings.
In the international iPCD cohort, we identified lobectomised and non-lobectomised age and sex-matched PCD patients and compared their characteristics, lung function and BMI cross-sectionally and longitudinally.
Among 2896 patients in the iPCD cohort, 163 from 20 centers (15 countries) underwent lung resection (5.6%). Among adult patients, prevalence of lung resection was 8.9%, demonstrating wide variation among countries. Compared to the rest of the iPCD cohort, lobectomised patients were more often females, older at diagnosis, and more often had situs solitus. In about half of the cases (45.6%) lung resection was performed before presentation to specialized PCD centers for diagnostic work-up. Compared to controls (n = 197), lobectomised patients had lower FVC z-scores (- 2.41 vs - 1.35, p = 0.0001) and FEV1 z-scores (- 2.79 vs - 1.99, p = 0.003) at their first post-lung resection assessment. After surgery, lung function continued to decline at a faster rate in lobectomised patients compared to controls (FVC z-score slope: - 0.037/year Vs - 0.009/year, p = 0.047 and FEV1 z-score slope: - 0.052/year Vs - 0.033/year, p = 0.235), although difference did not reach statistical significance for FEV1. Within cases, females and patients with multiple lobe resections had lower lung function.
Prevalence of lung resection in PCD varies widely between countries, is often performed before PCD diagnosis and overall is more frequent in patients with delayed diagnosis. After lung resection, compared to controls most lobectomised patients have poorer and continuing decline of lung function despite lung resection. Further studies benefiting from prospective data collection are needed to confirm these findings.
Mots-clé
Adolescent, Adult, Body Mass Index, Case-Control Studies, Child, Child, Preschool, Ciliary Motility Disorders/surgery, Cohort Studies, Cross-Sectional Studies, Female, Forced Expiratory Volume, Humans, Longitudinal Studies, Lung/surgery, Male, Prevalence, Prospective Studies, Respiratory Function Tests, Treatment Outcome, Young Adult, Ciliary motility disorders (MeSH), Kartagener syndrome (MeSH), Lobectomy
Pubmed
Web of science
Open Access
Oui
Création de la notice
27/09/2019 9:55
Dernière modification de la notice
15/01/2021 8:10