The dyskinetic cilia syndrome in childhood. Modifications of ultrastructural patterns.
The syndrome of cilia dyskinesia is known as a heterogenous ciliary dysfunction caused by morphological defects of the dynein arms, the nexin links, the radial spokes and by the transposition of microtubules. Supernumerary tubules have been regarded as acquired morphological defects on the background of other bronchopathies. The report of a 9-year-old girl with the clinical signs of ciliary dyskinesis is considered to be an attribution to the clinical and pathological features of this syndrome. The girl's history of chronic bronchopulmonary infections and nasal polyposis resistant to therapy made her suspected to be ill of Kartagener's syndrome. The results of ultrastructural investigations of the mucosa from ciliated epithelium revealed a ciliary structural defect in the bronchi as well as in the nose and the sinuses with supernumerary microtubular doublets and singles, a decentration of the central tubules and shortened dynein arms. The regularity of the electron optical abnormalities implicates a systemic disorder of ciliated epithelium, which is to be summarized to the syndrome of cilia dyskinesis.
D001980:Bronchi / Q000473:pathology; D001991:Bronchitis / Q000473:pathology; D001996:Bronchopneumonia / Q000473:pathology; D002648:Child; D002923:Cilia / Q000648:ultrastructure*; D002925:Ciliary Motility Disorders / Q000473:pathology*; D005260:Female; D006801:Humans; D008173:Lung Diseases, Obstructive / Q000473:pathology; D009092:Mucous Membrane / Q000473:pathology; D009297:Nasal Mucosa / Q000473:pathology; D009298:Nasal Polyps / Q000473:pathology; D012220:Rhinitis / Q000473:pathology