ReviewMultidisciplinary respiratory medicine2018
Why, when and how to investigate primary ciliary dyskinesia in adult patients with bronchiectasis.
Review in Multidisciplinary respiratory medicine, 2018. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 17 papers.
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Who cites it
17 citing papers in PubMed, 42 citations in OpenAlex.
- Bronchiectasis and sinonasal diseases: a narrative review.ERJ open research · 2025Review
- Primary Ciliary Dyskinesia in Adult Bronchiectasis: Data from the German Bronchiectasis Registry PROGNOSIS.Chest · 2024Article
- A novel splicing mutation DNAH5 c.13,338 + 5G > C is involved in the pathogenesis of primary ciliary dyskinesia in a family with primary familial brain calcification.BMC pulmonary medicine · 2024Article
- The Precision Medicine Era of Bronchiectasis.American journal of respiratory and critical care medicine · 2024Article
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- ERS International Congress 2022: highlights from the Airway Diseases Assembly.ERJ open research · 2023Review
- Which side are they on? Diagnosing primary ciliary dyskinesias in low- or middle-income countries: A review and case series.African journal of thoracic and critical care medicine · 2023Review
- Precision medicine in bronchiectasis.Breathe (Sheffield, England) · 2021Review
- Diagnosis of primary ciliary dyskinesia: discrepancy according to different algorithms.ERJ open research · 2021Article
- Whole genome sequencing in the diagnosis of primary ciliary dyskinesia.BMC medical genomics · 2021Article
- Protease-Antiprotease Imbalance in Bronchiectasis.International journal of molecular sciences · 2021Review
- Is bronchiectasis really a disease?European respiratory review : an official journal of the European Respiratory Society · 2020Article
- Understanding Primary Ciliary Dyskinesia: Experience From a Mediterranean Diagnostic Reference Centre.Journal of clinical medicine · 2020Article
- Severe pulmonary disease in an adult primary ciliary dyskinesia population in Brazil.Scientific reports · 2019Article
- Get together to increase awareness in bronchiectasis: a report of the 2Multidisciplinary respiratory medicine · 2018Article
- 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
13 authors at 5 institutions in 3 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Bronchiectasis represents the final pathway of several infectious, genetic, immunologic or allergic disorders. Accurate and prompt identification of the underlying cause is a key recommendation of several international guidelines, in order to tailor treatment appropriately. Primary ciliary dyskinesia (PCD) is a genetic cause of bronchiectasis in which failure of motile cilia leads to poor mucociliary clearance. Due to poor ciliary function in other organs, individuals can suffer from chronic rhinosinusitis, otitis media and infertility. This paper explores the current literature describing why, when and how to investigate PCD in adult patients with bronchiectasis. We describe the main PCD diagnostic tests and compare the two international PCD diagnostic guidelines. The expensive multi-test diagnostic approach requiring a high level of expertise and specialist equipment, make the multifaceted PCD diagnostic pathway complex. Therefore, the risk of late or missed diagnosis is high and has clinical and research implications. Defining the number of patients with bronchiectasis due to PCD is complex. To date, few studies outlining the aetiology of adult patients with bronchiectasis conduct screening tests for PCD, but they do differ in their diagnostic approach. Comparison of these studies reveals an estimated PCD prevalence of 1-13% in adults with bronchiectasis and describe patients as younger than their counterparts with moderate impairment of lung function and higher rates of chronic infection with
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