Evidence map›Paper›PMID 30723730›Full record

ArticleERJ open research2019

Pulmonary exacerbations in patients with primary ciliary dyskinesia: an expert consensus definition for use in clinical trials.

Jane S Lucas, Florian Gahleitner, Adelina Amorim, Mieke Boon, Philippa Brown, Carolina Constant, Simon Cook, Suzanne Crowley, Damien M S Destouches, Ernst Eber and 20 more

Open access · goldAbstract read
In one paragraph

Article in ERJ open research, 2019. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 35 papers, 1 of them a synthesis that pooled it.

0numbers the graph read from it
0cells of the map it votes in
35citing papers in PubMed, 1 pooled it
5.2field-weighted citation impact, top 4% 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

35 citing papers in PubMed, 1 synthesis or guideline pooled it, 80 citations in OpenAlex.

  1. Guideline
  2. Article
  3. Pulmonary exacerbations in primary ciliary dyskinesia.European journal of pediatrics · 2026
    Review
  4. Review
  5. Review
  6. Article
  7. Article
  8. Article
  9. Article
  10. Article
  11. Article
  12. Review
  13. Review
  14. Observational
  15. Article
  16. Review
  17. Article
  18. Article
  19. Review
  20. [Primary ciliary dyskinesia].Innere Medizin (Heidelberg, Germany) · 2024
    Review
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

30 authors at 20 institutions in 16 countries.

Jane S LucasPrimary Ciliary Dyskinesia Centre, NIHR Southampton Biomedical Research Centre, University of Southampton and University Hospital Southampton NHS Foundation Trust, Southampton, UK.ORCID https://orcid.org/0000-0001-8701-9975
Florian GahleitnerPrimary Ciliary Dyskinesia Centre, NIHR Southampton Biomedical Research Centre, University of Southampton and University Hospital Southampton NHS Foundation Trust, Southampton, UK.
Adelina AmorimPulmonology Dept, Centro Hospitalar S. João, Porto, Portugal.
Mieke BoonDept of Paediatrics, University Hospital Gasthuisberg, Leuven, Belgium.
Philippa BrownPCD Family Support Group, UK.
Carolina ConstantPaediatric Pulmonology Unit, Paediatrics Dept, Centro Hospitalar Lisboa Norte, Lisbon Academic Medical Centre, Lisbon, Portugal.ORCID https://orcid.org/0000-0002-6596-1596
Simon CookPCD Family Support Group, UK.
Suzanne CrowleyPaediatric Dept of Allergy and Lung Diseases, Oslo University Hospital, Oslo, Norway.
Damien M S DestouchesAssociation des Patients Ayant une Dyskinésie Ciliaire Primitive, France.
Ernst EberDivision of Paediatric Pulmonology and Allergology, Dept of Paediatrics and Adolescent Medicine, Medical University of Graz, Graz, Austria.
Huda MussaffiSchneider Children's Medical Center of Israel, Petach-Tikva and Sackler School of Medicine, Tel Aviv University, Tel Aviv, Israel.
Eric HaarmanDept of Pediatric Pulmonology, VU University Medical Center, Amsterdam, The Netherlands.
Amanda HarrisPrimary Ciliary Dyskinesia Centre, NIHR Southampton Biomedical Research Centre, University of Southampton and University Hospital Southampton NHS Foundation Trust, Southampton, UK.
Cordula Koerner-RettbergDept of Paediatric Pneumology, University Children's Hospital of Ruhr University, Bochum, Germany.
Claudia E KuehniInstitute of Social and Preventive Medicine, University of Bern, Bern, Switzerland.ORCID https://orcid.org/0000-0001-8957-2002
Philipp LatzinPaediatric Respiratory Medicine, University Children's Hospital of Bern, University of Bern, Bern, Switzerland.ORCID https://orcid.org/0000-0002-5239-1571
Michael R LoebingerHost Defence Unit, Royal Brompton and Harefield NHS Foundation Trust, London, UK.
Natalie LorentDept of Respiratory Medicine, University Hospitals Leuven, Leuven, Belgium.
Bernard MaitreService de Pneumologie, Hôpital Intercommunal de Créteil, DHU A-TVB, Inserm U955, Université Paris Est-Créteil, Créteil, France.
Antonio Moreno-GaldóSection of Pediatric Allergy, Pulmonology and Cystic Fibrosis, Hospital Vall d'Hebron, Universitat Autónoma de Barcelona, CIBERER, ISCIII, Barcelona, Spain.
Kim G NielsenDanish PCD & chILD Centre, CF Centre Copenhagen Paediatric Pulmonary Service, ERN Accredited for PCD and CF Health Care, Dept of Paediatrics and Adolescent Medicine, Copenhagen University Hospital, Rigshospitalet, Copenhagen, Denmark.
Uğur ÖzçelikDept of Pediatric Pulmonology, University Medical Faculty, Ankara, Turkey.
Lue Katrine Drasbæk PhilipsenDanish PCD & chILD Centre, CF Centre Copenhagen Paediatric Pulmonary Service, ERN Accredited for PCD and CF Health Care, Dept of Paediatrics and Adolescent Medicine, Copenhagen University Hospital, Rigshospitalet, Copenhagen, Denmark.
Petr PohunekPaediatric Dept, Second Faculty of Medicine, Charles University and Motol University Hospital, Prague, Czech Republic.
Eva PolverinoVall d'Hebron Research Institute, Respiratory Disease Dept, Hospital Vall d'Hebron, CIBER, Barcelona, Spain.
Jessica RademacherDept of Respiratory Medicine, Hannover Medical School, Hanover, Germany.
Phil RobinsonPCD Service, Dept of Respiratory and Sleep Medicine, Royal Children's Hospital, Melbourne, Australia.
Deborah SnijdersPrimary Ciliary Dyskinesia Centre, Dept of Woman and Child Health (SDB), University of Padova, Padua, Italy.
Panayiotis YiallourosMedical School, University of Cyprus, Nicosia, Cyprus.
Siobhán B CarrPrimary Ciliary Dyskinesia Centre, Dept of Paediatric Respiratory Medicine, Imperial College and Royal Brompton Hospital, London, UK.ORCID https://orcid.org/0000-0003-0580-2478
University Hospital Southampton NHS Foundation Trust · GBCopenhagen University Hospital · DKUniversity of Bern · CHAmsterdam UMC Location Vrije Universiteit Amsterdam · NLAnkara University · TRCentro Hospitalar Lisboa Norte · PTCharles University · CZInserm · FRMedical University of Graz · ATMedizinische Hochschule Hannover · DEOslo University Hospital · NORoyal Brompton & Harefield NHS Foundation Trust · GBRoyal Brompton Hospital · GBRoyal Children's Hospital · AUTel Aviv University · ILUniversidade do Porto · PTUniversitat Autònoma de Barcelona · ESUniversity Hospitals of the Ruhr-University of Bochum · DEUniversity of Cyprus · CYUniversity of Padua · IT

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

Pulmonary exacerbations are a cause of significant morbidity in patients with primary ciliary dyskinesia (PCD) and are frequently used as an outcome measure in clinical research into chronic lung diseases. So far, there has been no consensus on the definition of pulmonary exacerbations in PCD. 30 multidisciplinary experts and patients developed a consensus definition for children and adults with PCD. Following a systematic review, the panel used a modified Delphi process with a combination of face-to-face meetings and e-surveys to develop a definition that can be used in research settings for children and adults with PCD. A pulmonary exacerbation was defined by the presence of three or more of the following seven items: 1) increased cough, 2) change in sputum volume and/or colour, 3) increased shortness of breath perceived by the patient or parent, 4) decision to start or change antibiotic treatment because of perceived pulmonary symptoms, 5) malaise, tiredness, fatigue or lethargy, 6) new or increased haemoptysis, and 7) temperature >38°C. The consensus panel proposed that the definition should be used for future clinical trials. The definition should be validated and the usability assessed during these studies.

Identifiers

PMID30723730
PMCPMC6355979
OpenAlexW2911609816

What OpenQuestion holds

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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.