Pulmonary Exacerbations and Treatment in PCD: A Narrative Review.
A comprehensive review identifies major gaps in treating lung infections in primary ciliary dyskinesia, highlighting urgent research needs.
This narrative review synthesizes current knowledge on pulmonary exacerbations (PEx) in Primary Ciliary Dyskinesia (PCD), covering PEx definition variability, frequency, pathophysiology including airway inflammatory mediators, and risk factors such as female sex, age, and Pseudomonas aeruginosa infection. Treatment and prevention evidence remains scarce, emphasizing the research gap in managing this progressive rare lung disease with no approved disease-modifying therapies.
What the study was
- Study design
- Narrative review
- Population
- Patients with Primary Ciliary Dyskinesia (PCD) experiencing pulmonary exacerbations
- Category
- Treatment Innovation
- Maturity
- Exploratory
- Journal
- Pediatric pulmonology
Why it surfaced
Pediatric Pulmonology narrative review on PCD pulmonary exacerbations; PCD is a rare genetic disease with no approved disease-modifying therapies; review covers understudied treatment domain; Anagnostopoulou/Yiallouros (Cyprus) and Thee (Charité Berlin, German Lung Research Center).
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