Evidence map›Paper›PMID 30813645›Full record

ReviewGenes2019

Progression of Cystic Fibrosis Lung Disease from Childhood to Adulthood: Neutrophils, Neutrophil Extracellular Trap (NET) Formation, and NET Degradation.

Meraj A Khan, Zubair Sabz Ali, Neil Sweezey, Hartmut Grasemann, Nades Palaniyar

Open access · goldAbstract readReview
In one paragraph

Review in Genes, 2019. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 64 papers, 1 of them a synthesis that pooled it.

0numbers the graph read from it
0cells of the map it votes in
64citing papers in PubMed, 1 pooled it
5.4field-weighted citation impact, top 3% 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

64 citing papers in PubMed, 1 synthesis or guideline pooled it, 111 citations in OpenAlex.

  1. Pooled it
  2. Calcium regulation ofApplied and environmental microbiology · 2026
    Article
  3. Review
  4. Article
  5. Role of theInternational journal of molecular sciences · 2025
    Article
  6. Review
  7. Article
  8. Review
  9. Article
  10. Review
  11. Article
  12. Review
  13. Review
  14. Review
  15. Article
  16. Article
  17. Article
  18. American journal of physiology. Lung cellular and molecular physiology · 2024
    Article
  19. A crucial role of neutrophil extracellular traps in pulmonary infectious diseases.Chinese medical journal pulmonary and critical care medicine · 2024
    Review
  20. Article

4 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

5 authors at 2 institutions in 1 country.

Meraj A KhanTranslational Medicine, Peter Gilgan Center for Research and Learning, The Hospital for Sick Children, Toronto, ON M5G 0A4, Canada. meraj.khan@sickkids.ca.
Zubair Sabz AliTranslational Medicine, Peter Gilgan Center for Research and Learning, The Hospital for Sick Children, Toronto, ON M5G 0A4, Canada. zubair.ali@sickkids.ca.
Neil SweezeyTranslational Medicine, Peter Gilgan Center for Research and Learning, The Hospital for Sick Children, Toronto, ON M5G 0A4, Canada. neil.sweezey@sickkids.ca.
Hartmut GrasemannTranslational Medicine, Peter Gilgan Center for Research and Learning, The Hospital for Sick Children, Toronto, ON M5G 0A4, Canada. hartmut.grasemann@sickkids.ca.
Nades PalaniyarTranslational Medicine, Peter Gilgan Center for Research and Learning, The Hospital for Sick Children, Toronto, ON M5G 0A4, Canada. nades.palaniyar@sickkids.ca.
Hospital for Sick Children · CASickKids Foundation · CA

Funding

CIHR SVB 145590
6 · The paper itself

Abstract

Genetic defects in cystic fibrosis (CF) transmembrane conductance regulator (CFTR) gene cause CF. Infants with CFTR mutations show a peribronchial neutrophil infiltration prior to the establishment of infection in their lung. The inflammatory response progressively increases in children that include both upper and lower airways. Infectious and inflammatory response leads to an increase in mucus viscosity and mucus plugging of small and medium-size bronchioles. Eventually, neutrophils chronically infiltrate the airways with biofilm or chronic bacterial infection. Perpetual infection and airway inflammation destroy the lungs, which leads to increased morbidity and eventual mortality in most of the patients with CF. Studies have now established that neutrophil cytotoxins, extracellular DNA, and neutrophil extracellular traps (NETs) are associated with increased mucus clogging and lung injury in CF. In addition to opportunistic pathogens, various aspects of the CF airway milieux (e.g., airway pH, salt concentration, and neutrophil phenotypes) influence the NETotic capacity of neutrophils. CF airway milieu may promote the survival of neutrophils and eventual pro-inflammatory aberrant NETosis, rather than the anti-inflammatory apoptotic death in these cells. Degrading NETs helps to manage CF airway disease; since DNAse treatment release cytotoxins from the NETs, further improvements are needed to degrade NETs with maximal positive effects. Neutrophil-T cell interactions may be important in regulating viral infection-mediated pulmonary exacerbations in patients with bacterial infections. Therefore, clarifying the role of neutrophils and NETs in CF lung disease and identifying therapies that preserve the positive effects of neutrophils, while reducing the detrimental effects of NETs and cytotoxic components, are essential in achieving innovative therapeutic advances.

Indexed as

AdultCystic FibrosisCystic Fibrosis Transmembrane Conductance RegulatorCytotoxinsDisease ProgressionExtracellular TrapsHumansInfantLung DiseasesNeutrophilsCFTR protein, humanCystic Fibrosis Transmembrane Conductance RegulatorCytotoxinscystic fibrosiscystic fibrosis transmembrane conductance regulatorDNaselung diseaseNETosisneutrophil extracellular traps (NETs)neutrophils

Identifiers

PMID30813645
PMCPMC6471578
OpenAlexW2918027232

What OpenQuestion holds

Textmetadata
LicenceCC BY
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.