Evidence map›Paper›PMID 27836958›Full record

GuidelineThe European respiratory journal2017

European Respiratory Society guidelines for the diagnosis of primary ciliary dyskinesia.

Jane S Lucas, Angelo Barbato, Samuel A Collins, Myrofora Goutaki, Laura Behan, Daan Caudri, Sharon Dell, Ernst Eber, Estelle Escudier, Robert A Hirst and 22 more

3 registry-linked trialsAbstract readPractice Guideline
In one paragraph

Guideline in The European respiratory journal, 2017. The graph could read no effect estimate from its abstract, so it casts no vote on the map. It is linked to 3 registered trials, which are not on this map. Cited by 323 papers, 4 of them syntheses that pooled it.

0numbers the graph read from it
0cells of the map it votes in
323citing papers in PubMed, 4 pooled it
–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.

NCT07148362 completednot on this mapstarted 2021, after this paper: background citation

Comparison of Upper Extremity Exercise Capacity, Muscle Oxygenation, and Balance in Pediatric Cystic Fibrosis and Primary Ciliary Dyskinesia

TypeobservationalSponsorGazi UniversityRan2021 to 2025Enrolled88ConditionsCystic Fibrosis (CF), Primary Ciliary Dyskinesia (PCD)
NCT07157644 completednot on this mapstarted 2021, after this paper: background citation

Comparison of Functional Exercise Capacity, Muscle Oxygenation, Respiratory Muscle Strength, and Physical Activity Level in Pediatric Cystic Fibrosis and Primary Ciliary Dyskinesia

TypeobservationalSponsorGazi UniversityRan2021 to 2025Enrolled88ConditionsCystic Fibrosis (CF), Primary Ciliary Dyskinesia (PCD)
NCT07566611 narecruitingnot on this mapstarted 2026, after this paper: background citation

Novel Use of Combined High-Speed Video Microscopy and Nasal Nitric Oxide Screening for Identification of Primary Ciliary Dyskinesia in Adult Bronchiectasis

TypeinterventionalSponsorIndiana UniversityRan2026 to 2028Enrolled60ConditionsIdiopathic BronchiectasisArmsNasal Nitric Oxide Measurement, Nasopharyngeal (Nasal) Samples
3 · Its place in the literature

Who cites it

323 citing papers in PubMed, 4 syntheses or guidelines pooled it.

  1. Guideline
  2. Guideline
  3. Pooled it
  4. Pooled it
  5. Trial
  6. Trial
  7. Article
  8. Article
  9. Review
  10. Article
  11. Article
  12. Article
  13. Article
  14. Review
  15. Utilising human cellular models of primary ciliary dyskinesia: a scoping review.European respiratory review : an official journal of the European Respiratory Society · 2026
    Article
  16. Article
  17. Article
  18. Article
  19. Article
  20. [Report of 24 cases of primary ciliary dyskinesia in children].Zhongguo dang dai er ke za zhi = Chinese journal of contemporary pediatrics · 2026
    Article

263 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

32 authors.

Jane S LucasPrimary Ciliary Dyskinesia Centre, University Hospital Southampton NHS Foundation Trust, Southampton, UK jlucas1@soton.ac.uk.
Angelo BarbatoPrimary Ciliary Dyskinesia Centre, Dept of Woman and Child Health (SDB), University of Padova, Padova, Italy.
Samuel A CollinsPrimary Ciliary Dyskinesia Centre, University Hospital Southampton NHS Foundation Trust, Southampton, UK.ORCID 0000-0003-1571-7410
Myrofora GoutakiInstitute of Social and Preventive Medicine, University of Bern, Bern, Switzerland.
Laura BehanPrimary Ciliary Dyskinesia Centre, University Hospital Southampton NHS Foundation Trust, Southampton, UK.
Daan CaudriTelethon Kids Institute, The University of Western Australia, Subiaco, Australia.
Sharon DellDivision of Respiratory Medicine, The Hospital for Sick Children, Toronto, ON, Canada.
Ernst EberDivision of Paediatric Pulmonology and Allergology, Dept of Paediatrics and Adolescent Medicine, Medical University of Graz, Graz, Austria.
Estelle EscudierService de Génétique et Embryologie Médicales, Centre de Référence des Maladies Respiratoires Rares, Hôpital Armand Trousseau, Assistance Publique - Hôpitaux de Paris (AP-HP), Paris, France.
Robert A HirstCentre for PCD Diagnosis and Research, Dept of Infection, Immunity and Inflammation, University of Leicester, Leicester Royal Infirmary, Leicester, UK.
Claire HoggDepts of Paediatrics and Paediatric Respiratory Medicine, Imperial College and Royal Brompton Hospital, London, UK.
Mark JorissenENT Dept, University Hospitals Leuven, Leuven, Belgium.
Philipp LatzinDept of Paediatrics, Inselspital, University Hospital of Bern, University of Bern, Bern, Switzerland.
Marie LegendreService de Génétique et Embryologie Médicales, Centre de Référence des Maladies Respiratoires Rares, Hôpital Armand Trousseau, Assistance Publique - Hôpitaux de Paris (AP-HP), Paris, France.
Margaret W LeighUniversity of North Carolina at Chapel Hill, Chapel Hill, NC, USA.
Fabio MidullaPaediatric Dept, Sapienza University of Rome, Rome, Italy.
Kim G NielsenDanish PCD & chILD Centre, CF Centre Copenhagen, Paediatric Pulmonary Service, Dept of Paediatrics and Adolescent Medicine, Copenhagen University Hospital, Rigshospitalet, Copenhagen, Denmark.
Heymut OmranDept of Pediatrics, University Hospital Muenster, Münster Germany.
Jean-Francois PaponAP-HP, Hôpital Kremlin-Bicetre, service d'ORL et de chirurgie cervico-faciale, Le Kremlin-Bicetre, France.
Petr PohunekPaediatric Dept, Second Faculty of Medicine, Charles University and Motol University Hospital, Prague, Czech Republic.
Beatrice RedfernPCD Family Support Group, Milton Keynes, UK.
David RigauIberoamerican Cochrane Center, Barcelona, Spain.
Bernhard RindlisbacherKartagener Syndrom und Primäre Ciliäre Dyskinesie e. V., Steffisburg, Switzerland.
Francesca SantamariaPediatric Pulmonology, Dept of Translational Medical Sciences, Federico II University, Azienda Ospedaliera Universitaria Federico II, Naples, Italy.
Amelia ShoemarkDepts of Paediatrics and Paediatric Respiratory Medicine, Imperial College and Royal Brompton Hospital, London, UK.
Deborah SnijdersPrimary Ciliary Dyskinesia Centre, Dept of Woman and Child Health (SDB), University of Padova, Padova, Italy.
Thomy ToniaInstitute of Social and Preventive Medicine, University of Bern, Bern, Switzerland.
Andrea TitieniDept of Pediatrics, University Hospital Muenster, Münster Germany.
Woolf T WalkerPrimary Ciliary Dyskinesia Centre, University Hospital Southampton NHS Foundation Trust, Southampton, UK.
Claudius WernerDept of Pediatrics, University Hospital Muenster, Münster Germany.
Andrew BushDepts of Paediatrics and Paediatric Respiratory Medicine, Imperial College and Royal Brompton Hospital, London, UK.
Claudia E KuehniInstitute of Social and Preventive Medicine, University of Bern, Bern, Switzerland.

Funding

Longitudinal Characterization of Respiratory Tract Exacerbations and Treatment Responses in Primary Ciliary DyskinesiaU54HL096458 · NHLBI · UNIV OF NORTH CAROLINA CHAPEL HILL · PI DAVIS, STEPHANIE DUGGINS, FERKOL, THOMAS W · 2009 to 2023
$20.0M
Department of Health NIHR-HCS-P13-04-004NHLBI NIH HHS U54 HL096458
6 · The paper itself

Abstract

The diagnosis of primary ciliary dyskinesia is often confirmed with standard, albeit complex and expensive, tests. In many cases, however, the diagnosis remains difficult despite the array of sophisticated diagnostic tests. There is no "gold standard" reference test. Hence, a Task Force supported by the European Respiratory Society has developed this guideline to provide evidence-based recommendations on diagnostic testing, especially in light of new developments in such tests, and the need for robust diagnoses of patients who might enter randomised controlled trials of treatments. The guideline is based on pre-defined questions relevant for clinical care, a systematic review of the literature, and assessment of the evidence using the GRADE (Grading of Recommendations, Assessment, Development and Evaluation) approach. It focuses on clinical presentation, nasal nitric oxide, analysis of ciliary beat frequency and pattern by high-speed video-microscopy analysis, transmission electron microscopy, genotyping and immunofluorescence. It then used a modified Delphi survey to develop an algorithm for the use of diagnostic tests to definitively confirm and exclude the diagnosis of primary ciliary dyskinesia; and to provide advice when the diagnosis was not conclusive. Finally, this guideline proposes a set of quality criteria for future research on the validity of diagnostic methods for primary ciliary dyskinesia.

Indexed as

CiliaDelphi TechniqueDiagnosis, DifferentialEuropeFluorescent Antibody TechniqueGenetic TestingHumansKartagener SyndromeMicroscopy, Electron, TransmissionMicroscopy, VideoNitric OxideReview Literature as TopicSocieties, MedicalNitric Oxide

Identifiers

PMID27836958
PMCPMC6054534

What OpenQuestion holds

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Registered trials

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.