ArticleRespiratory research2019
Prevalence and course of disease after lung resection in primary ciliary dyskinesia: a cohort & nested case-control study.
Article in Respiratory research, 2019. The graph could read no effect estimate from its abstract, so it casts no vote on the map. It is linked to trial NCT03517865 (International Primary Ciliary Dyskinesia Cohort), which is not on this map. Cited by 12 papers.
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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.
International Primary Ciliary Dyskinesia Cohort
Who cites it
12 citing papers in PubMed, 28 citations in OpenAlex.
- 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
- Primary ciliary dyskinesia: a national expert consensus statement on standards of care.ERJ open research · 2026Article
- The RaDiCo information system for rare disease cohorts.Orphanet journal of rare diseases · 2025Article
- Lung Transplantation for Primary Ciliary Dyskinesia and Kartagener Syndrome: A Multicenter Study.Transplant international : official journal of the European Society for Organ Transplantation · 2023Article
- Lung function from school age to adulthood in primary ciliary dyskinesia.The European respiratory journal · 2022Article
- Facemask Usage Among People With Primary Ciliary Dyskinesia During the COVID-19 Pandemic: A Participatory Project.International journal of public health · 2021Observational
- COVID-PCD: a participatory research study on the impact of COVID-19 in people with primary ciliary dyskinesia.ERJ open research · 2021Article
- Motile ciliopathies.Nature reviews. Disease primers · 2020Review
- Late Diagnosis of Infants with PCD and Neonatal Respiratory Distress.Journal of clinical medicine · 2020Article
- Registries and collaborative studies for primary ciliary dyskinesia in Europe.ERJ open research · 2020Review
- Proceedings of the 4BMC proceedings · 2020Article
- The Primary Ciliary Dyskinesia Computed Tomography Score in Adults with Bronchiectasis: A Derivation und Validation Study.Respiration; international review of thoracic diseasesArticle
Corrections and comments
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Authors and funding
35 authors at 16 institutions in 15 countries.
Funding
Abstract
backgroundLung resection is a controversial and understudied therapeutic modality in Primary Ciliary Dyskinesia (PCD). We assessed the prevalence of lung resection in PCD across countries and compared disease course in lobectomised and non-lobectomised patients.
methodsIn the international iPCD cohort, we identified lobectomised and non-lobectomised age and sex-matched PCD patients and compared their characteristics, lung function and BMI cross-sectionally and longitudinally.
resultsAmong 2896 patients in the iPCD cohort, 163 from 20 centers (15 countries) underwent lung resection (5.6%). Among adult patients, prevalence of lung resection was 8.9%, demonstrating wide variation among countries. Compared to the rest of the iPCD cohort, lobectomised patients were more often females, older at diagnosis, and more often had situs solitus. In about half of the cases (45.6%) lung resection was performed before presentation to specialized PCD centers for diagnostic work-up. Compared to controls (n = 197), lobectomised patients had lower FVC z-scores (- 2.41 vs - 1.35, p = 0.0001) and FEV1 z-scores (- 2.79 vs - 1.99, p = 0.003) at their first post-lung resection assessment. After surgery, lung function continued to decline at a faster rate in lobectomised patients compared to controls (FVC z-score slope: - 0.037/year Vs - 0.009/year, p = 0.047 and FEV1 z-score slope: - 0.052/year Vs - 0.033/year, p = 0.235), although difference did not reach statistical significance for FEV1. Within cases, females and patients with multiple lobe resections had lower lung function.
conclusionsPrevalence of lung resection in PCD varies widely between countries, is often performed before PCD diagnosis and overall is more frequent in patients with delayed diagnosis. After lung resection, compared to controls most lobectomised patients have poorer and continuing decline of lung function despite lung resection. Further studies benefiting from prospective data collection are needed to confirm these findings.
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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.