Evidence map›Paper›PMID 37845149›Full record

Observational studyJournal of cystic fibrosis : official journal of the European Cystic Fibrosis Society2024

Effect of elexacaftor/tezacaftor/ivacaftor on mucus and mucociliary clearance in cystic fibrosis.

Scott H Donaldson, Timothy E Corcoran, Joseph M Pilewski, Peter Mogayzel, Beth L Laube, Evan R Boitet, Elex S Harris, Agathe Ceppe, Lloyd J Edwards, Kirby Zeman and 5 more

Open access · bronzeAbstract readObservational Study
In one paragraph

Observational study in Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society, 2024. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 22 papers.

0numbers the graph read from it
0cells of the map it votes in
22citing papers in PubMed
7.0field-weighted citation impact, top 2% of its field
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

22 citing papers in PubMed, 27 citations in OpenAlex.

  1. Trial
  2. Article
  3. Article
  4. Differential Evolution ofPathogens (Basel, Switzerland) · 2026
    Article
  5. Review
  6. Observational
  7. Article
  8. Airway mucus in infection.Frontiers in physiology · 2026
    Review
  9. Article
  10. Observational
  11. Article
  12. ERJ open research · 2025
    Article
  13. Article
  14. State-of-the-Art Review: Transformative Changes in the Care of People With Cystic Fibrosis: Implications for Infectious Diseases Specialists.Clinical infectious diseases : an official publication of the Infectious Diseases Society of America · 2025
    Review
  15. Article
  16. Review
  17. Article
  18. The lysogenic filamentousPNAS nexus · 2024
    Article
  19. Cystic fibrosis.Nature reviews. Disease primers · 2024
    Review
  20. 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

15 authors at 5 institutions in 1 country.

Scott H DonaldsonDepartment of Medicine, Univ. North Carolina at Chapel Hill, USA. Electronic address: scott_donaldson@med.unc.edu.
Timothy E CorcoranPulmonary, Allergy and Critical Care Medicine, University of Pittsburgh, USA.
Joseph M PilewskiPulmonary, Allergy and Critical Care Medicine, University of Pittsburgh, USA.
Peter MogayzelPediatric Pulmonary Medicine, Johns Hopkins University, USA.
Beth L LaubePediatric Pulmonary Medicine, Johns Hopkins University, USA.
Evan R BoitetDepartment of Medicine and the Gregory Fleming James Cystic Fibrosis Research Center, University of Alabama at Birmingham, USA.
Elex S HarrisDepartment of Medicine and the Gregory Fleming James Cystic Fibrosis Research Center, University of Alabama at Birmingham, USA.
Agathe CeppeDepartment of Medicine, Univ. North Carolina at Chapel Hill, USA.
Lloyd J EdwardsDepartment of Biostatistics, University of Alabama at Birmingham, USA.
Kirby ZemanDepartment of Medicine, Univ. North Carolina at Chapel Hill, USA.
Jihong WuDepartment of Medicine, Univ. North Carolina at Chapel Hill, USA.
Charles R EstherPediatric Pulmonology, Department of Pediatrics, Univ. North Carolina at Chapel Hill, USA.
David P NicholsDepartment of Pediatrics, University of Washington School of Medicine, USA.
William D BennettDepartment of Medicine, Univ. North Carolina at Chapel Hill, USA.
Steven M RoweDepartment of Medicine and the Gregory Fleming James Cystic Fibrosis Research Center, University of Alabama at Birmingham, USA.
University of North Carolina at Chapel Hill · USUniversity of Alabama at Birmingham · USJohns Hopkins University · USUniversity of Pittsburgh · USUniversity of Washington · US

Funding

QAQC Johns Hopkins Institute for Clinical and Translational ResearchUL1TR003098 · NCATS · JOHNS HOPKINS UNIVERSITY · PI FORD, DANIEL ERNEST · 2019 to 2023
$57.7M
UNC-CH CENTER FOR ENVIRONMENTAL HEALTH &SUSCEPTIBILITYP30ES010126 · NIEHS · UNIV OF NORTH CAROLINA CHAPEL HILL · PI Hazel B Nichols · 2001 to 2026
$36.3M
Vector CoreP30DK065988 · NIDDK · UNIV OF NORTH CAROLINA CHAPEL HILL · PI Scott H Randell · 2004 to 2026
$26.5M
UAB CF Research and Translation Core CenterP30DK072482 · NIDDK · UNIVERSITY OF ALABAMA AT BIRMINGHAM · PI AMIT GAGGAR · 2007 to 2026
$23.0M
NCATS NIH HHS UL1 TR003098NIDDK NIH HHS P30 DK065988NIDDK NIH HHS P30 DK072482NIEHS NIH HHS P30 ES010126
6 · The paper itself

Abstract

backgroundThe cystic fibrosis transmembrane conductance regulator (CFTR) modulator elexacaftor/tezacaftor/ivacaftor (E/T/I) is highly effective clinically for those with at least one F508del-CFTR allele. The effects of E/T/I on mucociliary clearance (MCC) and sputum properties are unknown. We, therefore, sought to characterize the effects of E/T/I on in vivo MCC and sputum characteristics hypothesized to impact mucus transport.

methodsForty-four participants ≥12 years of age were enrolled into this prospective, observational trial prior to initiation of E/T/I and had baseline measurement of MCC and characterization of induced sputum and exhaled breath condensate (EBC) samples. Study procedures were repeated after 1 month of E/T/I treatment.

resultsAverage age was 27.7 years with baseline forced expiratory volume in 1 second (FEV

conclusionsE/T/I improved the hydration of respiratory secretions (% solids) and markedly accelerated MCC. These data confirm the link between CFTR function, mucus solid content, and MCC and help to define the utility of MCC and mucus-related bioassays in future efforts to restore CFTR function in all people with CF.

Indexed as

Cystic FibrosisIndolesPyrazolesPyridinesPyrrolidinesQuinolonesAdultAminophenolsBenzodioxolesChloride Channel AgonistsCystic Fibrosis Transmembrane Conductance RegulatorHumansMucociliary ClearanceMucusMutationProspective StudiesAminophenolsBenzodioxolesChloride Channel AgonistsCystic Fibrosis Transmembrane Conductance RegulatorelexacaftorIndolesivacaftorPyrazolesPyridinesPyrrolidinesQuinolonestezacaftorCystic fibrosisElexacaftorModulatorMucociliary clearancemucusRheology

Identifiers

PMID37845149
PMCPMC10948316
OpenAlexW4387643246

What OpenQuestion holds

Textmetadata
LicenceTDM
Read underepoch 390

Registered trials

None linked

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.