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Pulmonary exacerbations in patients with genetically confirmed PCD: A prospective observational multicentre study
2025-11-19
respiratory medicine
Title + abstract only
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BackgroundPrimary ciliary dyskinesia (PCD) is a rare genetic disorder characterized by deficient mucociliary clearance and development of chronic lung disease. Pulmonary exacerbations (PEx) in chronic lung diseases increase morbidity and lung function decline, but their frequency in PCD has been understudied. We aimed to prospectively determine the annual frequency of PEx in PCD and identify related risk factors. MethodsIn a multicentre, observational study conducted in 11 centres from seven co...
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