ArticleERJ open research2026
Incidence and predictors of self-reported pulmonary exacerbations in primary ciliary dyskinesia: an international prospective cohort study.
Article in ERJ open research, 2026. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 5 papers.
What it found
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Who cites it
5 citing papers in PubMed.
- Pulmonary exacerbations in primary ciliary dyskinesia.European journal of pediatrics · 2026Review
- Airway Clearance and Pediatric Pulmonary Rehabilitation in Primary Ciliary Dyskinesia: A Clinical Framework for Children and Adolescents.Journal of clinical medicine · 2026Review
- Pulmonary Exacerbations and Treatment in PCD: A Narrative Review.Pediatric pulmonology · 2026Review
- Pulmonary exacerbations in patients with genetically confirmed PCD: a prospective observational multicentre study.Frontiers in molecular biosciences · 2026Article
- Beyond the present: current and future perspectives on the role of infections in pediatric PCD.Frontiers in pediatrics · 2025Review
Corrections and comments
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Authors and funding
8 authors.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Introduction: Pulmonary exacerbations contribute to disease progression in chronic lung diseases. In a large prospective cohort study, we studied the incidence and predictors of pulmonary exacerbations among persons with primary ciliary dyskinesia (PCD), which can inform follow-up care. We also assessed healthcare use, changes in management and pathogens during exacerbations. Methods: Participants in the Living with PCD study reported increased respiratory symptoms in the past 7 days, indicating a pulmonary exacerbation, from June 2020 through May 2022 Results: We obtained data from 660 persons (408 adults, 57 adolescents, 195 children) who completed 17 853 follow-up questionnaires (median 17, range 1-84). The 1026 reported exacerbations indicate an incidence rate of 3.1 pulmonary exacerbations per person per year, with minor variation across age groups, but changes over time. Incidence was higher among adult females (incidence rate ratio (IRR) 2.0, 95% confidence interval (CI) 1.4-2.7) and those in whom Conclusion: Pulmonary exacerbations are frequent in PCD and heighten the disease burden. Patients for whom targeted management is particularly important include adult females and those who carry
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Registered trials
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