Article
Airway Disease in Children with Primary Ciliary Dyskinesia: Impact of Ciliary Ultrastructure Defect and Genotype.
Annals of the American Thoracic Society - 1 Apr 2023
Kinghorn BreAnna, Rosenfeld Margaret, Sullivan Erin, Onchiri Frankline, Ferkol Thomas W, Sagel Scott D, Dell Sharon D, Milla Carlos, Shapiro Adam J, Sullivan Kelli M, Zariwala Maimoona A, Pittman Jessica E, Mollica Federico, Tiddens Harm A W M, Kemner-van de Corput Mariette, Knowles Michael R, Davis Stephanie D, Leigh Margaret W
Abstract excerpt
Rationale: Primary ciliary dyskinesia (PCD) is characterized by impaired mucociliary clearance, recurrent respiratory infections, progressive airway damage, and obstructive lung disease. Although the association of ciliary ultrastructure defect/genotype with the severity of airflow obstruction has been well characterized, their association with airway abnormalities on chest computed tomography (CT) has been...
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