Evidence map›Paper›PMID 30944166›Full record

ArticleScience translational medicine2019

Mucus accumulation in the lungs precedes structural changes and infection in children with cystic fibrosis.

Charles R Esther, Marianne S Muhlebach, Camille Ehre, David B Hill, Matthew C Wolfgang, Mehmet Kesimer, Kathryn A Ramsey, Matthew R Markovetz, Ian C Garbarine, M Gregory Forest and 11 more

Open access · greenAbstract read
In one paragraph

Article in Science translational medicine, 2019. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 119 papers.

0numbers the graph read from it
0cells of the map it votes in
119citing papers in PubMed
19.5field-weighted citation impact, top 1% 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

119 citing papers in PubMed, 193 citations in OpenAlex.

  1. Trial
  2. Trial
  3. Article
  4. Review
  5. Article
  6. Article
  7. Article
  8. Structural and cellular mechanisms of mucus plugging in the larger airways.Chinese medical journal pulmonary and critical care medicine · 2026
    Review
  9. Article
  10. Review
  11. Article
  12. Hyperconcentrated mucus in small airways: a mechanistic model for the pathogenesis of paediatric bronchiectasis.European respiratory review : an official journal of the European Respiratory Society · 2025
    Review
  13. Article
  14. Article
  15. Article
  16. Review
  17. Insights on the Pathogenesis ofJournal of clinical medicine · 2025
    Review
  18. Article
  19. Review
  20. Review

59 more citing papers are in PubMed but not listed here.

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

21 authors at 5 institutions in 2 countries.

Charles R EstherDivision of Pediatric Pulmonology, University of North Carolina at Chapel Hill, Chapel Hill, NC 27599, USA. charles_esther@med.unc.edu.ORCID 0000-0002-8081-2986
Marianne S MuhlebachDivision of Pediatric Pulmonology, University of North Carolina at Chapel Hill, Chapel Hill, NC 27599, USA.ORCID 0000-0001-6391-1598
Camille EhreDivision of Pediatric Pulmonology, University of North Carolina at Chapel Hill, Chapel Hill, NC 27599, USA.ORCID 0000-0002-0046-0096
David B HillMarsico Lung Institute, University of North Carolina at Chapel Hill, Chapel Hill, NC 27599, USA.
Matthew C WolfgangMarsico Lung Institute, University of North Carolina at Chapel Hill, Chapel Hill, NC 27599, USA.
Mehmet KesimerMarsico Lung Institute, University of North Carolina at Chapel Hill, Chapel Hill, NC 27599, USA.
Kathryn A RamseyMarsico Lung Institute, University of North Carolina at Chapel Hill, Chapel Hill, NC 27599, USA.ORCID 0000-0003-4574-6917
Matthew R MarkovetzMarsico Lung Institute, University of North Carolina at Chapel Hill, Chapel Hill, NC 27599, USA.ORCID 0000-0003-3931-0594
Ian C GarbarineMarsico Lung Institute, University of North Carolina at Chapel Hill, Chapel Hill, NC 27599, USA.
M Gregory ForestDepartments of Mathematics, Biomedical Engineering, and Applied Physical Sciences, University of North Carolina at Chapel Hill, Chapel Hill, NC 27599, USA.
Ian SeimDepartments of Mathematics, Biomedical Engineering, and Applied Physical Sciences, University of North Carolina at Chapel Hill, Chapel Hill, NC 27599, USA.ORCID 0000-0001-9719-2552
Bryan ZornMarsico Lung Institute, University of North Carolina at Chapel Hill, Chapel Hill, NC 27599, USA.
Cameron B MorrisonMarsico Lung Institute, University of North Carolina at Chapel Hill, Chapel Hill, NC 27599, USA.ORCID 0000-0002-4031-9521
Martial F DelionMarsico Lung Institute, University of North Carolina at Chapel Hill, Chapel Hill, NC 27599, USA.
William R ThelinParion Sciences, Durham, NC 27713, USA.ORCID 0000-0001-9083-9632
Diane VillalonParion Sciences, Durham, NC 27713, USA.
Juan R SabaterDepartment of Research, Mount Sinai Medical Center, Miami Beach, FL 33140, USA.
Lidija TurkovicTelethon Kids Institute, University of Western Australia, Perth 6009, Australia.
Sarath RanganathanMurdoch Children's Research Institute, University of Melbourne, Parkville 3052, Australia.
Stephen M StickTelethon Kids Institute, University of Western Australia, Perth 6009, Australia.ORCID 0000-0002-5386-8482
Richard C BoucherMarsico Lung Institute, University of North Carolina at Chapel Hill, Chapel Hill, NC 27599, USA.
University of North Carolina at Chapel Hill · USParion Sciences (United States) · USThe Kids Research Institute Australia · AUMount Sinai Medical Center · USThe University of Melbourne · AU

Funding

UNC-CH CENTER FOR ENVIRONMENTAL HEALTH &SUSCEPTIBILITYP30ES010126 · NIEHS · UNIV OF NORTH CAROLINA CHAPEL HILL · PI Stephanie Engel · 2001 to 2026
$36.3M
Vector CoreP30DK065988 · NIDDK · UNIV OF NORTH CAROLINA CHAPEL HILL · PI Martina Gentzsch · 2004 to 2026
$26.5M
The Biophysics of Mucus Hydration and Adhesion/CohesionP01HL108808 · NHLBI · UNIV OF NORTH CAROLINA CHAPEL HILL · PI BOUCHER, RICHARD CHARLES, PEDEN, DAVID B. · 2012 to 2021
$23.9M
Purinergic Control of CFTR-ENaC Interactions in Alveolar EpitheliaP01HL110873 · NHLBI · UNIV OF NORTH CAROLINA CHAPEL HILL · PI BOUCHER, RICHARD CHARLES · 2012 to 2016
$10.7M
Synthesis of Effective and Safe Mucolytics for Pulmonary DiseaseUH3HL123645 · NHLBI · UNIV OF NORTH CAROLINA CHAPEL HILL · PI BOUCHER, RICHARD CHARLES · 2016 to 2018
$4.6M
Multiscale Biochemical/Biophysical Integration of Pulmonary Mucus TransportR01HL136961 · NHLBI · UNIV OF NORTH CAROLINA CHAPEL HILL · PI BOUCHER, RICHARD CHARLES · 2017 to 2020
$3.2M
Mucus and hypoxia in heterogeneous and progressive CF lung diseaseR01HL116228 · NHLBI · UNIV OF NORTH CAROLINA CHAPEL HILL · PI ESTHER, CHARLES RICHARD, MUHLEBACH, MARIANNE S · 2012 to 2015
$2.1M
Mucus Adhesion to Epithelial Cells and its Mobilization in COPD and CFP50HL107168 · NHLBI · UNIV OF NORTH CAROLINA CHAPEL HILL · PI BOUCHER, RICHARD CHARLES, DOERSCHUK, CLAIRE M · 2011 to 2012
$885k
Purines as Biomarkers of Respiratory DiseaseK23HL089708 · NHLBI · UNIV OF NORTH CAROLINA CHAPEL HILL · PI ESTHER, CHARLES RICHARD · 2009 to 2013
$685k
NHLBI NIH HHS K23 HL089708NHLBI NIH HHS P01 HL108808NHLBI NIH HHS P01 HL110873NHLBI NIH HHS P50 HL107168NHLBI NIH HHS R01 HL116228NHLBI NIH HHS R01 HL136961NHLBI NIH HHS UH3 HL123645NIDDK NIH HHS P30 DK065988NIEHS NIH HHS P30 ES010126
6 · The paper itself

Abstract

Although destructive airway disease is evident in young children with cystic fibrosis (CF), little is known about the nature of the early CF lung environment triggering the disease. To elucidate early CF pulmonary pathophysiology, we performed mucus, inflammation, metabolomic, and microbiome analyses on bronchoalveolar lavage fluid (BALF) from 46 preschool children with CF enrolled in the Australian Respiratory Early Surveillance Team for Cystic Fibrosis (AREST CF) program and 16 non-CF disease controls. Total airway mucins were elevated in CF compared to non-CF BALF irrespective of infection, and higher densities of mucus flakes containing mucin 5B and mucin 5AC were observed in samples from CF patients. Total mucins and mucus flakes correlated with inflammation, hypoxia, and oxidative stress. Many CF BALFs appeared sterile by culture and molecular analyses, whereas other samples exhibiting bacterial taxa associated with the oral cavity. Children without computed tomography-defined structural lung disease exhibited elevated BALF mucus flakes and neutrophils, but little/no bacterial infection. Although CF mucus flakes appeared "permanent" because they did not dissolve in dilute BALF matrix, they could be solubilized by a previously unidentified reducing agent (P2062), but not

Indexed as

AnimalsBiomarkersCase-Control StudiesChildChild, PreschoolCystic FibrosisFemaleHumansInfantInflammationLungMaleMicrobiotaMucusSheepBiomarkers

Identifiers

PMID30944166
PMCPMC6566903
OpenAlexW2932017142

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.