Evidence map›Paper›PMID 42427487›Full record

ArticleFrontiers in molecular biosciences2026

Pulmonary exacerbations in patients with genetically confirmed PCD: a prospective observational multicentre study.

Pinelopi Anagnostopoulou, Panayiotis Kouis, Dilber Ademhan Tural, Sarah Altaraihi, Simona Basilicata, Melissa Borrelli, Athina Christofidou, Hatice Nur Coemert, Renato Cutrera, Nagehan Emiralioglou and 23 more

Abstract read
In one paragraph

Article in Frontiers in molecular biosciences, 2026. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 2 papers.

0numbers the graph read from it
0cells of the map it votes in
2citing papers in PubMed
–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.

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

2 citing papers in PubMed.

  1. Pulmonary exacerbations in primary ciliary dyskinesia.European journal of pediatrics · 2026
    Review
  2. 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

33 authors.

Pinelopi AnagnostopoulouMedical School, University of Cyprus, Nicosia, Cyprus.
Panayiotis KouisMedical School, University of Cyprus, Nicosia, Cyprus.
Dilber Ademhan TuralSchool of Medicine, Hacettepe University, Ankara, Turkey.
Sarah AltaraihiDanish PCD Centre Copenhagen, Paediatric Pulmonary Service, Copenhagen University Hospital, Copenhagen, Denmark.
Simona BasilicataFederico II University, Naples, Italy.
Melissa BorrelliFederico II University, Naples, Italy.
Athina ChristofidouMedical School, University of Cyprus, Nicosia, Cyprus.
Hatice Nur CoemertDanish PCD Centre Copenhagen, Paediatric Pulmonary Service, Copenhagen University Hospital, Copenhagen, Denmark.
Renato CutreraBambino Gesù Children's Hospital, IRCCS, Rome, Italy.
Nagehan EmiralioglouSchool of Medicine, Hacettepe University, Ankara, Turkey.
Ela ErdemMarmara University School of Medicine, Istanbul, Turkey.
Yasemin GokdemirMarmara University School of Medicine, Istanbul, Turkey.
Serena GracciUniversity Hospital of Pisa, Pisa, Italy.
Elpis HatziagorouAristotle University of Thessaloniki, Thessaloniki, Greece.
Simone HelmsUniversity Hospital Muenster, Muenster, Germany.
Bülent KaradagMarmara University School of Medicine, Istanbul, Turkey.
Debora MajUniversity Hospital of Pisa, Pisa, Italy.
Marcus A MallDepartment of Pediatric Respiratory Medicine, Immunology and Critical Care Medicine, Charité-Universitätsmedizin Berlin, Berlin, Germany.
June K MarthinDanish PCD Centre Copenhagen, Paediatric Pulmonary Service, Copenhagen University Hospital, Copenhagen, Denmark.
Nicos MiddletonCyprus University of Technology, Limassol, Cyprus.
Antonio Moreno-GaldóDepartment of Pediatrics, Vall D'Hebron Hospital Universitari, Vall D'Hebron Barcelona Hospital Campus, Universitat Autònoma de Barcelona, Barcelona, Spain and CIBER of Rare Diseases (CIBERER), Instituto de Salud Carlos III (ISCIII), Madrid, Spain.
Ugur ÖzçelikSchool of Medicine, Hacettepe University, Ankara, Turkey.
Massimo PifferiUniversity Hospital of Pisa, Pisa, Italy.
Johanna RaidtUniversity Hospital Muenster, Muenster, Germany.
Jobst RöhmelDepartment of Pediatric Respiratory Medicine, Immunology and Critical Care Medicine, Charité-Universitätsmedizin Berlin, Berlin, Germany.
Sandra Rovira-AmigoDepartment of Pediatrics, Vall D'Hebron Hospital Universitari, Vall D'Hebron Barcelona Hospital Campus, Universitat Autònoma de Barcelona, Barcelona, Spain and CIBER of Rare Diseases (CIBERER), Instituto de Salud Carlos III (ISCIII), Madrid, Spain.
Francesca SantamariaFederico II University, Naples, Italy.
Ioannis TsiolakisMedical School, University of Cyprus, Nicosia, Cyprus.
Nicola UllmannBambino Gesù Children's Hospital, IRCCS, Rome, Italy.
Niklas ZiegahnDepartment of Pediatric Respiratory Medicine, Immunology and Critical Care Medicine, Charité-Universitätsmedizin Berlin, Berlin, Germany.
Kim G NielsenDanish PCD Centre Copenhagen, Paediatric Pulmonary Service, Copenhagen University Hospital, Copenhagen, Denmark.
Heymut OmranUniversity Hospital Muenster, Muenster, Germany.
Panayiotis YiallourosMedical School, University of Cyprus, Nicosia, Cyprus.

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

Background: Primary ciliary dyskinesia (PCD) is a rare genetic disorder characterized by deficient mucociliary clearance and development of chronic lung disease. Pulmonary exacerbations (PEx) in chronic lung diseases increase morbidity and lung function decline, but their frequency in PCD has been understudied. We aimed to prospectively determine the annual frequency of PEx in PCD and identify related risk factors. Methods: In a multicentre, observational study conducted in 11 centres from seven countries, we prospectively collected data from a well-described patient cohort with genetically confirmed PCD over a year via monthly telephone questionnaires and clinical visits. We assessed the annual PEx frequency using three definitions: i) clinical definition 1 ( Results: We recruited 271 individuals with PCD of all ages. Among patients with complete annual records (n = 248), approximately 80% experienced at least one PEx per year, as assessed by the three definitions used. Self-reported PEx per year (median 2, interquartile range (IQR) 1-5) were higher (p < 0.0001) than the PEx assessed by Def-1 (median 2, IQR 0-4) and Def-2 (median 1, IQR 0.25-3). Self-reported PEx had a substantial agreement with Def-1 [kappa (SE) = 0.61 (0.05)] and a moderate agreement with Def-2 [kappa (SE) = 0.51 (0.05)]. Female sex and autumn season were associated with higher number of PEx, independent of the definition used. Increasing age was correlated with higher annual PEx frequency by Def-1. Conclusion: In this multicentre study, we prospectively assessed the annual PEx frequency in patients genetically diagnosed with PCD, demonstrating the importance of the definition used in capturing the exacerbation burden of PCD, as well as the influence of sex, age and season on exacerbation frequency.

Indexed as

cohortmulticentre studyprimary ciliary dyskinesiapulmonary exacerbationsquestionnairerare disease

Identifiers

PMID42427487
PMCPMC13345937

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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.