ArticleChest2024
Primary Ciliary Dyskinesia in Adult Bronchiectasis: Data from the German Bronchiectasis Registry PROGNOSIS.
Article in Chest, 2024. The graph could read no effect estimate from its abstract, so it casts no vote on the map. It is linked to trial NCT02574143 (The PROspective German NOn-CF bronchiectaSIS Patient Registry), which is not on this map. Cited by 14 papers, 2 of them syntheses that pooled it.
What it found
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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.
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.
The PROspective German NOn-CF bronchiectaSIS Patient Registry
Who cites it
14 citing papers in PubMed, 2 syntheses or guidelines pooled it.
- Coexistence of COPD or asthma in patients with bronchiectasis: a systematic review and meta-analysis.BMC pulmonary medicine · 2026Pooled it
- Nutrition and growth of primary ciliary dyskinesia patients: a systematic review.European respiratory review : an official journal of the European Respiratory Society · 2026Pooled it
- Nasal nitric oxide combined with clinical features for preliminary screening of primary ciliary dyskinesia in bronchiectasis.The European respiratory journal · 2026Article
- The frequent exacerbator phenotype in bronchiectasis revisited: data from EMBARC registry.American journal of respiratory and critical care medicine · 2026Article
- Pulmonary Exacerbations and Treatment in PCD: A Narrative Review.Pediatric pulmonology · 2026Review
- Association of German Bronchiectasis Registry participation with disease course.ERJ open research · 2026Article
- PCDSOS: a novel clinical predictive tool for screening primary ciliary dyskinesia in adult bronchiectasis patients-a multicenter derivation and external validation study.BMC medicine · 2026Article
- Inflammatory Endotypes of Chronic Rhinosinusitis and Impacts on Comorbid Asthma.Journal of inflammation research · 2026Review
- Greater disease severity in adults with paediatric-onsetThe European respiratory journal · 2026Article
- Allergic bronchopulmonary aspergillosis andJournal of thoracic disease · 2025Review
- ODAD4-Related Primary Ciliary Dyskinesia: Report of Five Cases and a Founder Variant in Quebec.Cells · 2025Article
- Gene expression profiling of airway epithelium inERJ open research · 2025Article
- [Physiotherapy in Primary Ciliary Dyskinesia].Pneumologie (Stuttgart, Germany) · 2025Review
- Primary ciliary dyskinesia: Aetiology, diagnosis and clinical management.Clinical medicine (London, England) · 2025Review
Corrections and comments
PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.
Authors and funding
13 authors.
Funding
No grant is acknowledged in the PubMed record.
Abstract
backgroundPrimary ciliary dyskinesia (PCD) is a rare genetic disorder caused by the malfunction of motile cilia and a specific etiology of adult bronchiectasis of unknown prevalence. A better understanding of the clinical phenotype of adults with PCD is needed to identify individuals for referral to diagnostic testing. RESEARCH QUESTION: What is the frequency of PCD among adults with bronchiectasis; how do people with PCD differ from those with other etiologies; and which clinical characteristics are independently associated with PCD? STUDY DESIGN AND
methodsWe investigated the proportion of PCD among the participants of the Prospective German Non-CF-Bronchiectasis Registry (PROGNOSIS) study; applied multiple imputation to account for missing data in 64 (FEV
resultsWe consecutively recruited 1,000 patients from 38 centers across all levels of the German health care system. Overall, PCD was the fifth most common etiology of bronchiectasis in 87 patients (9%) after idiopathic, postinfective, COPD, and asthma. People with PCD showed a distinct clinical phenotype. In multivariate regression analysis, the chance of PCD being the etiology of bronchiectasis increased with the presence of upper airway disease (chronic rhinosinusitis and/or nasal polyps; adjusted OR [aOR], 6.3; 95% CI, 3.3-11.9; P < .001), age < 53 years (aOR, 5.3; 95% CI, 2.7-10.4; P < .001), radiologic involvement of any middle and lower lobe (aOR, 3.7; 95% CI, 1.3-10.8; P = .016), duration of bronchiectasis > 15 years (aOR, 3.6; 95% CI, 1.9-6.9; P < .001), and a history of Pseudomonas aeruginosa isolation from respiratory specimen (aOR, 2.4; 95% CI, 1.3-4.5; P = .007).
interpretationWithin our nationally representative cohort, PCD was a common etiology of bronchiectasis. We identified few easy-to-assess phenotypic features, which may promote awareness for PCD among adults with bronchiectasis. CLINICAL
trial registrationClinicalTrials.gov; No.: NCT02574143; URL: www. CLINICALTRIALS: gov.
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