Primary ciliary dyskinesia (PCD), formerly known as immotile cilia syndrome, is a disorder of motile cilia structure and function that results in chronic oto-sinopulmonary disease. Primary ciliary dyskinesia typically presents with respiratory distress in infants, early onset year-round cough, and nasal congestion.
Immotile cilia syndrome (ICS) is an autosomal recessive disease with extensive genetic heterogeneity characterized by abnormal ciliary motion and impaired mucociliary clearance. Ultrastructural and functional defects of cilia result in the lack of effective ciliary motility, causing abnormal mucociliary clearance.
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