Evidence map›Paper›PMID 41846681›Full record

ArticleERJ open research2026

Long-term impact of elexacaftor/tezacaftor/ivacaftor on small and large airways in people with cystic fibrosis aged ≥6 years: 24-month real-world evidence from the German Cystic Fibrosis Registry.

Stefanie Dillenhöfer, Katharina Schütz, Manuel Burkhart, Helmut Ellemunter, Matthias Kappler, Sarah Sieber, Lutz Naehrlich, Folke Brinkmann, Sivagurunathan Sutharsan

Erratum issuedAbstract read
In one paragraph

Article in ERJ open research, 2026. The graph could read no effect estimate from its abstract, so it casts no vote on the map. An erratum has been issued. Cited by 2 papers.

0numbers the graph read from it
0cells of the map it votes in
2citing 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

2 citing papers in PubMed.

  1. Article
  2. Article
4 · The record

Corrections and comments

5 · Who and what money

Authors and funding

9 authors.

Stefanie DillenhöferDepartment of Pediatric Pulmonology, Cystic Fibrosis Center, University Children's Hospital of Ruhr University Bochum at St. Josef-Hospital, Bochum, Germany.ORCID https://orcid.org/0000-0001-6020-8717
Katharina SchützClinic of Paediatric Pneumology, Allergology and Neonatology, Hannover Medical School and Biomedical Research in Endstage and Obstructive Lung Disease Hannover (BREATH), Member of the German Center for Lung Research (DZL), Hannover, Germany.ORCID https://orcid.org/0000-0003-2680-3541
Manuel BurkhartMukoviszidose Institut gGmbH, Bonn, Germany.
Helmut EllemunterMedical University of Innsbruck, Cystic Fibrosis Centre Innsbruck, Innsbruck, Austria.
Matthias KapplerDepartment of Pediatrics, Dr von Hauner Children's Hospital, University Hospital, LMU Munich, Munich, Germany.
Sarah SieberSTAT-UP Statistical Consulting and Data Science GmbH, Munich, Germany.
Lutz NaehrlichDepartment of Pediatrics, Justus-Liebig-University Giessen, Giessen, Germany.
Folke BrinkmannDepartment of Pediatric Pneumology and Allergology, The University of Lübeck, University Medical Center Schleswig-Holstein, Campus Centrum Lübeck, Member of Airway Research Center North of the DZL, Lübeck, Germany.
Sivagurunathan SutharsanDepartment of Pulmonary Medicine, University Hospital Essen - Ruhrlandklinik, Adult Cystic Fibrosis Center, University of Duisburg-Essen, Essen, Germany.

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

Background: While studies have consistently reported improvements in lung function during elexacaftor/tezacaftor/ivacaftor (ETI) therapy for people with cystic fibrosis (pwCF), individual response predictors remain poorly understood and there is limited real-world data relating to children aged 6-11 years. Methods: Lung function data from the German CF Registry 2020-2024 were analysed before and up to 24 months after ETI therapy initiation in adults, adolescents and children aged ≥6 years. Data were stratified for percent predicted forced expiratory volume in 1 s (ppFEV Findings: Data from 2375 pwCF were analysed. After starting ETI, ppFEV Interpretation: Our real-world data showed significant improvements in lung function, including large and small airways, during 24 months' ETI therapy in pwCF aged ≥6 years. Notable improvements, particularly in small airways, were observed even in children and adolescents with normal ppFEV

Identifiers

PMID41846681
PMCPMC12991016

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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.