title: Ciliary ultrastructure in patients with chronic rhinosinusitis and primary ciliary dyskinesia.
abstract: The Cilia represent one of the main mechanisms contributing to the clearance of microorganisms and particles from the respiratory epithelium. Primary ciliary dyskinesia (PCD) is a genetically determined disorder characterized by irreversible systemic dysmotility of the cilia. Secondary ciliary dyskinesia (SCD) differs from primary defects on the reversible ultrastructural alterations that can occur after any insult to a previously normal mucosa. Hence, this study aimed to describe and compare the main ultrastructural ciliary features in PCD and SCD through transmission electron microscopy. The most frequent PCD abnormalities were missing or short dynein arms, missing central microtubules, and displacement of one of the nine peripheral doublets. The most common changes found in SCD were compound cilia and peripheral microtubule alterations associated with modifications of the respiratory epithelium. PCD presented a higher percentage of altered cilia (>30 %) when compared to SCD (5 %), demonstrating that SCD is more limited in area than PCD. Whereas in PCD the changes in the dynein arms and in the central microtubules are fundamental for diagnostic confirmation, the diagnosis of SCD usually involves compound cilia and disarrangements in peripheral microtubules.
authors.affiliation: Department of Ophthalmology, School of Medicine of Ribeirão Preto, University of São Paulo, Av Bandeirantes, 3900, Ribeirão Preto, SP 14049-900, Brazil.
authors.forename: Ricardo Cassiano
authors.initials: RC
authors.lastname: Demarco
authors.forename: Edwin
authors.initials: E
authors.lastname: Tamashiro
authors.forename: Maria
authors.initials: M
authors.lastname: Rossato
authors.forename: Maria Dolores Seabra
authors.initials: MD
authors.lastname: Ferreira
authors.forename: Fabiana Cardoso Pereira
authors.initials: FC
authors.lastname: Valera
authors.forename: Wilma T
authors.initials: WT
authors.lastname: Anselmo-Lima
mesh_terms: D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D002908:Chronic Disease; D002923:Cilia / Q000648:ultrastructure*; D002925:Ciliary Motility Disorders / Q000473:pathology* / Q000503:physiopathology; D003937:Diagnosis, Differential; D005260:Female; D006801:Humans; D007619:Kartagener Syndrome / Q000473:pathology* / Q000503:physiopathology; D008297:Male; D046529:Microscopy, Electron, Transmission; D020545:Respiratory Mucosa / Q000648:ultrastructure*; D012852:Sinusitis / Q000235:genetics / Q000473:pathology* / Q000503:physiopathology