Scanning and transmission electron microscopic aspects of the nasal acilia syndrome.
A 3-month-old boy was admitted with failure to thrive and persistent fevers. During a 4 month hospitalization for treatment of suspected sepsis, persistent purulent nasal discharge developed. Biopsies of his nasal mucosa on 3 separate occasions disclosed thinned respiratory epithelium and a complete absence of cilia when examined by electron microscopy (EM). Despite an initial granulocytopenia and a wide range in T-cell numbers, he did not show any evidence of lower respiratory tract infection. A tracheal biopsy process for EM demonstrated normal ciliated epithelium. This patient appears to have an unrecognized syndrome of normal tracheal cilia but absent nasal cilia.
D002675:Child, Preschool; D002923:Cilia / Q000648:ultrastructure*; D006801:Humans; D007223:Infant; D008297:Male; D008854:Microscopy, Electron; D008855:Microscopy, Electron, Scanning; D009297:Nasal Mucosa / Q000648:ultrastructure*; D009668:Nose Diseases / Q000151:congenital* / Q000473:pathology; D010859:Pigmentation Disorders / Q000151:congenital; D012871:Skin Diseases / Q000151:congenital*; D013577:Syndrome