Lung structural and functional impairments in young children with cystic fibrosis diagnosed following newborn screening - A nationwide observational study.

Children Cystic fibrosis Lung clearance index Magnetic resonance imaging Multiple breath washout

Journal

Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society
ISSN: 1873-5010
Titre abrégé: J Cyst Fibros
Pays: Netherlands
ID NLM: 101128966

Informations de publication

Date de publication:
25 Jun 2024
Historique:
received: 19 12 2023
revised: 04 04 2024
accepted: 20 05 2024
medline: 27 6 2024
pubmed: 27 6 2024
entrez: 26 6 2024
Statut: aheadofprint

Résumé

Non-invasive and sensitive clinical endpoints are needed to monitor onset and progression of early lung disease in children with cystic fibrosis (CF). We compared lung clearance index (LCI), FEV Lung function (LCI, FEV LCI, ventilation and perfusion defects, and structural MRI scores were significantly higher in children with CF compared with controls, but FEV The LCI and lung MRI outcomes non-invasively detect even mild early lung disease in young children with CF diagnosed following newborn screening. Pulmonary exacerbations and early respiratory symptoms were risk factors for structural and functional impairment in childhood.

Sections du résumé

BACKGROUND BACKGROUND
Non-invasive and sensitive clinical endpoints are needed to monitor onset and progression of early lung disease in children with cystic fibrosis (CF). We compared lung clearance index (LCI), FEV
METHODS METHODS
Lung function (LCI, FEV
RESULTS RESULTS
LCI, ventilation and perfusion defects, and structural MRI scores were significantly higher in children with CF compared with controls, but FEV
CONCLUSIONS CONCLUSIONS
The LCI and lung MRI outcomes non-invasively detect even mild early lung disease in young children with CF diagnosed following newborn screening. Pulmonary exacerbations and early respiratory symptoms were risk factors for structural and functional impairment in childhood.

Identifiants

DOI: 10.1016/j.jcf.2024.05.010 PMID: 38926017
pubmed: 38926017
pii: S1569-1993(24)00074-2
doi: 10.1016/j.jcf.2024.05.010
pii:
doi:

Types de publication

Journal Article

Langues

eng

Sous-ensembles de citation

IM

Informations de copyright

Copyright © 2024 The Authors. Published by Elsevier B.V. All rights reserved.

Déclaration de conflit d'intérêts

Declaration of competing interest The authors declare the following financial interests/personal relationships which may be considered as potential competing interests: Prof. P. Latzin reports the followind COIs: - Grants from Vertex and OM Pharma paid to the institution - Participation on data safety monitoring boards or advisory boards of Polyphor, Santhera DMC, Vertex, OM Pharma, Vifor, Sanofi Aventis - Payment or honoraria for lectures, presentations, speakers bureaus, manuscript writing or educational events: Vertex, Vifor, OM Pharma Jakob Usemann reports: Grants from - Swiss lung foundation - Palatin Foundation, Basel, Switzerland Payment or honoraria for lectures, presentations, speakers bureaus, manuscript writing or educational events: - Vertex - Zurich Lung foundation Support for attending meetings and/or travel from Vertex Kathryn Ramsey reports: Support for the present manuscript form: -Swiss National Science Foundation Ambizione Research Grant (168173), paid to the institution Leadership or fiduciary role in other board, society, committee or advocacy group, unpaid Global Lung Initiative MBW Task Force Elisabeth Kieninger reports: Payment or honoraria for lectures, presentations, speakers bureaus, manuscript writing or educational events: Sanofi-Aventis.

Auteurs

Bettina S Frauchiger (BS)

Pediatric Respiratory Medicine, Department of Pediatrics, Inselspital, Bern University Hospital, University of Bern, Bern, Switzerland.

Corin Willers (C)

Pediatric Respiratory Medicine, Department of Pediatrics, Inselspital, Bern University Hospital, University of Bern, Bern, Switzerland; Department of Paediatrics, Kantonsspital Aarau, Aarau, Switzerland.

Jasna Cotting (J)

Pediatric Respiratory Medicine, Department of Pediatrics, Inselspital, Bern University Hospital, University of Bern, Bern, Switzerland.

Elisabeth Kieninger (E)

Pediatric Respiratory Medicine, Department of Pediatrics, Inselspital, Bern University Hospital, University of Bern, Bern, Switzerland.

Insa Korten (I)

Pediatric Respiratory Medicine, Department of Pediatrics, Inselspital, Bern University Hospital, University of Bern, Bern, Switzerland.

Carmen Casaulta (C)

Pediatric Respiratory Medicine, Department of Pediatrics, Inselspital, Bern University Hospital, University of Bern, Bern, Switzerland.

Yasmin Salem (Y)

Pediatric Respiratory Medicine, Department of Pediatrics, Inselspital, Bern University Hospital, University of Bern, Bern, Switzerland.

Enno Stranzinger (E)

Diagnostic, interventional and pediatric radiology, Department of Pediatrics, Inselspital, Bern University Hospital, University of Bern, Bern, Switzerland.

Ben Brabandt (B)

Diagnostic, interventional and pediatric radiology, Department of Pediatrics, Inselspital, Bern University Hospital, University of Bern, Bern, Switzerland.

Jakob Usemann (J)

Division of Respiratory Medicine and Children's Research Center, University Children's Hospital Zurich, Zurich, Switzerland; University Children's Hospital Basel (UKBB), Basel, Switzerland.

Nicolas Regamey (N)

Department of Respiratory Medicine, Children's Hospital Luzern, Luzern, Switzerland.

Alena Kuhn (A)

Department of Paediatrics, Kantonsspital Aarau, Aarau, Switzerland.

Sylvain Blanchon (S)

Hôpital de l'enfance de Lausanne, Lausanne Switzerland.

Isabelle Rochat (I)

Hôpital de l'enfance de Lausanne, Lausanne Switzerland.

Grzegorz Bauman (G)

Division of Radiological Physics, Department of Radiology, University of Basel Hospital, Basel, Switzerland; Department of Biomedical Engineering, University of Basel, Allschwil, Switzerland.

Dominik Müller-Suter (D)

Department of Paediatrics, Kantonsspital Aarau, Aarau, Switzerland.

Alexander Moeller (A)

Division of Respiratory Medicine and Children's Research Center, University Children's Hospital Zurich, Zurich, Switzerland.

Philipp Latzin (P)

Pediatric Respiratory Medicine, Department of Pediatrics, Inselspital, Bern University Hospital, University of Bern, Bern, Switzerland.

Kathryn A Ramsey (KA)

Pediatric Respiratory Medicine, Department of Pediatrics, Inselspital, Bern University Hospital, University of Bern, Bern, Switzerland; Wal-yan Respiratory Research Centre, Telethon Kids Institute, University of Western Australia, Perth WA Australia. Electronic address: Kathryn.Ramsey@telethonkids.org.au.

Classifications MeSH