TL;DR
HGF levels in both serum and BAL fluid are significantly associated with disease severity in children with primary ciliary dyskinesia, and their associations with clinical, functional, and radiological measures of disease severity are investigated.
Variants in the CFTR and SFTPC genes may be associated with PCD in children, and this case highlights the importance of early genetic variant testing and ciliary ultrastructural analysis in children with recurrent respiratory tract infections, bronchiectasis, or chronic sinusitis.
Primary ciliary dyskinesia (PCD) is a rare inherited disorder of motile ciliary dysfunction characterized by impaired mucociliary clearance, chronic sino-pulmonary disease, and progressive bronchiectasis. Pulmonary exacerbations (PEx) are a major contributor to morbidity, lung function decline, and healthcare utilizati...
Background. Neutrophilic airway inflammation is a central pathogenic mechanism in bronchiectasis. However, the prognostic value of serum inflammatory biomarkers in the paediatric population remains uncertain, and most available evidence derives from mixed-etiology cohorts and adult patients. The aim of the study was to...
This review aims to synthesize emerging data to provide a primer on PCD, as well as summarize newer diagnostic approaches, and synthesize emerging data to provide a primer on PCD.
Primary ciliary dyskinesia (PCD) is an inherited motile-cilia disorder. Impaired mucociliary transport promotes persistent secretion retention, infection, inflammation, and bronchiectasis. The central treatment problem is therefore failure of airway clearance rather than infection alone. This narrative review evaluates...
Adult SRG-related ILD/PF is likely under-recognised because of variable penetrance, wide age at onset, and non-specific imaging features.
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