Lung clearance index to characterize clinical phenotypes of children and adolescents with cystic fibrosis.
Cystic fibrosis
Lung clearance index
Pediatrics
Phenotypes
Time-to-event data
Journal
BMC pulmonary medicine
ISSN: 1471-2466
Titre abrégé: BMC Pulm Med
Pays: England
ID NLM: 100968563
Informations de publication
Date de publication:
01 Apr 2022
01 Apr 2022
Historique:
received:
23
09
2021
accepted:
11
03
2022
entrez:
2
4
2022
pubmed:
3
4
2022
medline:
6
4
2022
Statut:
epublish
Résumé
Lung clearance index (LCI) is accepted as an early marker of lung disease in cystic fibrosis (CF), however the utility of LCI to identify subgroups of CF disease in the paediatric age group has never been explored. The aim of the study was to characterize phenotypes of children with CF using LCI as a marker of ventilation inhomogeneity and to investigate whether these phenotypes distinguished patients based on time to pulmonary exacerbation (PE). Data were collected on patients with CF aged < 18 years old, attending the CF Center of Milan during outpatient follow-up visits between October 2014 and September 2019. Cluster analysis using agglomerative nesting hierarchical method was performed to generate distinct phenotypes. Time-to-recurrent event analysis investigated association of phenotypes with PE. We collected 313 multiple breath washout tests on 125 children aged 5.5-16.8 years. Cluster analysis identified two divergent phenotypes in children and adolescents of same age, presenting with almost normal FEV LCI is useful in clinical practice to characterize distinct phenotypes of children and adolescents with mild/normal FEV
Sections du résumé
BACKGROUND
BACKGROUND
Lung clearance index (LCI) is accepted as an early marker of lung disease in cystic fibrosis (CF), however the utility of LCI to identify subgroups of CF disease in the paediatric age group has never been explored. The aim of the study was to characterize phenotypes of children with CF using LCI as a marker of ventilation inhomogeneity and to investigate whether these phenotypes distinguished patients based on time to pulmonary exacerbation (PE).
METHODS
METHODS
Data were collected on patients with CF aged < 18 years old, attending the CF Center of Milan during outpatient follow-up visits between October 2014 and September 2019. Cluster analysis using agglomerative nesting hierarchical method was performed to generate distinct phenotypes. Time-to-recurrent event analysis investigated association of phenotypes with PE.
RESULTS
RESULTS
We collected 313 multiple breath washout tests on 125 children aged 5.5-16.8 years. Cluster analysis identified two divergent phenotypes in children and adolescents of same age, presenting with almost normal FEV
CONCLUSIONS
CONCLUSIONS
LCI is useful in clinical practice to characterize distinct phenotypes of children and adolescents with mild/normal FEV
Identifiants
pubmed: 35365111
doi: 10.1186/s12890-022-01903-5
pii: 10.1186/s12890-022-01903-5
pmc: PMC8976307
doi:
Types de publication
Journal Article
Langues
eng
Sous-ensembles de citation
IM
Pagination
122Informations de copyright
© 2022. The Author(s).
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