Evidence map›Paper›PMID 40539675›Full record

ArticlePediatric pulmonology2025

Real-World Improvements of Lung Clearance Index and Ventilation Distribution Efficiency in Children With Cystic Fibrosis After Elexacaftor/Tezacaftor/Ivacaftor Initiation.

Esben Herborg Henriksen, Rikke Mulvad Sandvik, Mette Frahm Olsen, Tacjana Pressler, Thomas Bryrup, Christian Leo-Hansen, Bibi Uhre Nielsen, Christine Højte, Marianne Skov, Frederik Buchvald and 8 more

Abstract read
PubMed Full text at PMC Publisher
In one paragraph

Article in Pediatric pulmonology, 2025. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 1 paper.

0numbers the graph read from it
0cells of the map it votes in
1citing papers in PubMed
–field-weighted citation impact
1 · What the graph read from it

What it found

Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.

The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.

2 · The registry

The trial behind it

Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.

Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.

3 · Its place in the literature

Who cites it

1 citing paper in PubMed.

  1. Article
4 · The record

Corrections and comments

PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.

5 · Who and what money

Authors and funding

18 authors.

Esben Herborg HenriksenThe Danish Cystic Fibrosis Cohort, Copenhagen, Denmark.ORCID https://orcid.org/0000-0002-1247-8779
Rikke Mulvad SandvikDepartment of Pediatrics and Adolescent Medicine, Rigshospitalet, Copenhagen, Denmark.ORCID https://orcid.org/0000-0002-0374-7279
Mette Frahm OlsenThe Danish Cystic Fibrosis Cohort, Copenhagen, Denmark.
Tacjana PresslerThe Danish Cystic Fibrosis Cohort, Copenhagen, Denmark.
Thomas BryrupThe Danish Cystic Fibrosis Cohort, Copenhagen, Denmark.ORCID https://orcid.org/0000-0001-8533-6766
Christian Leo-HansenThe Danish Cystic Fibrosis Cohort, Copenhagen, Denmark.
Bibi Uhre NielsenThe Danish Cystic Fibrosis Cohort, Copenhagen, Denmark.
Christine HøjteThe Danish Cystic Fibrosis Cohort, Copenhagen, Denmark.
Marianne SkovThe Danish Cystic Fibrosis Cohort, Copenhagen, Denmark.
Frederik BuchvaldDepartment of Pediatrics and Adolescent Medicine, Rigshospitalet, Copenhagen, Denmark.
Inger Hee Mabuza MathiesenThe Danish Cystic Fibrosis Cohort, Copenhagen, Denmark.
Tavs QvistThe Danish Cystic Fibrosis Cohort, Copenhagen, Denmark.
Hanne Vebert OlesenThe Danish Cystic Fibrosis Cohort, Copenhagen, Denmark.
Sune RubakDepartment of Pediatrics and Adolescents Medicine, Danish Center of Pediatric Pulmonology and Allergology, University Hospital of Aarhus, Aarhus, Denmark.ORCID https://orcid.org/0000-0001-7010-2350
Camilla Bjørn JensenThe Danish Cystic Fibrosis Cohort, Copenhagen, Denmark.ORCID https://orcid.org/0000-0002-8224-4601
Daniel Faurholt-JepsenThe Danish Cystic Fibrosis Cohort, Copenhagen, Denmark.
Kim Gjerum NielsenDepartment of Pediatrics and Adolescent Medicine, Rigshospitalet, Copenhagen, Denmark.
TransformCF Study Group

Funding

The study was funded by Vertex Pharmaceuticals.
6 · The paper itself

Abstract

introductionElexacaftor/tezacaftor/ivacaftor (ETI) is a breakthrough therapy for cystic fibrosis (CF). We aimed to assess ETI's real-world impact on peripheral airway disease assessed as ventilation distribution inhomogeneity using nitrogen multiple breath washout (N

methodsThis nationwide study included data from N

resultsWe included 131 children (78% homozygous for F508del mutation, mean [SD] age 11.5 [3.4]), and 339 N

conclusionOur research demonstrates that ETI treatment significantly improves lung function, as measured by N

Indexed as

AminophenolsBenzodioxolesCystic FibrosisIndolesLungPyrazolesPyridinesPyrrolidinesQuinolonesAdolescentChildChloride Channel AgonistsDrug CombinationsFemaleHumansMaleAminophenolsBenzodioxolesChloride Channel AgonistsDrug Combinationselexacaftor, ivacaftor, tezacaftor drug combinationIndolesivacaftorPyrazolesPyridinesPyrrolidinesQuinolinesQuinolonescystic fibrosiselexacaftor/tezacaftor/ivacaftorLCIreal world studyVDE

Identifiers

PMID40539675
PMCPMC12180290

What OpenQuestion holds

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Registered trials

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Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.