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...

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Bibliographic Details
Published inThe Laryngoscope Vol. 92; no. 3; p. 297
Main Authors Dudley, J P, Welch, M J, Stiehm, E R, Carney, J M, Soderberg-Warner, M
Format Journal Article
LanguageEnglish
Published United States 01.03.1982
Subjects
Child, Preschool
Cilia - ultrastructure
Humans
Infant
Male
Microscopy, Electron
Microscopy, Electron, Scanning
Nasal Mucosa - ultrastructure
Nose Diseases - congenital
Nose Diseases - pathology
Pigmentation Disorders - congenital
Skin Diseases - congenital
Syndrome
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Summary: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.
ISSN:0023-852X
DOI:10.1288/00005537-198203000-00014