Ciliary dyskinesia in the nose and paranasal sinuses.
Primary ciliary dyskinesia is a rare autosomal recessive disorder of which 50% with situs inversus Kartagener's syndrome. Secondary ciliary dyskinesia is a frequent observation, mostly in association with or after respiratory tract infections. Diagnosis and differential diagnosis are mostly based on the typical clinical picture, the absence of mucociliary clearance and ciliary activity and the electron microscopical demonstration of ultrastructural abnormalities. However, these investigations are not always conclusive. Functional and ultrastructural ciliary evaluation after ciliogenesis in tissue culture is essential and crucial.
Jorissen
M
M
ENT Department, Head and Neck Surgery, UZ Sint Rafaël, Leuven, Belgium.
Bertrand
B
B
Eloy
P
P
D002923:Cilia / Q000502:physiology / Q000648:ultrastructure; D002925:Ciliary Motility Disorders / Q000175:diagnosis* / Q000209:etiology / Q000235:genetics / Q000473:pathology / Q000503:physiopathology; D003937:Diagnosis, Differential; D005808:Genes, Recessive; D006801:Humans; D007619:Kartagener Syndrome / Q000235:genetics; D008854:Microscopy, Electron; D009079:Mucociliary Clearance; D009297:Nasal Mucosa / Q000648:ultrastructure; D010256:Paranasal Sinuses / Q000648:ultrastructure; D012141:Respiratory Tract Infections / Q000150:complications