Primary ciliary dyskinesia: Diagnosis in children with inconclusive ultrastructural evaluation

The purpose of this study was to distinguish between acquired and genetically determined ciliary abnormalities in children with severe chronic respiratory diseases. Samples of nasal ciliated epithelium from 50 subjects (25 male, 25 female; age‐range 2–19 years) with severe chronic respiratory diseas...

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Published in:Pediatric allergy and immunology 2001-10, Vol.12 (5), p.274-282
Main Authors: Pifferi, Massimo, Cangiotti, Angela M., Ragazzo, Vincenzo, Baldini, Giuliano, Cinti, Saverio, Boner, Attilio L.
Format: Article
Language:English
Subjects:
Adolescent
Adult
Biological and medical sciences
Child
Child Welfare
Child, Preschool
children
Chronic obstructive pulmonary disease, asthma
chronic respiratory disease
Cilia - pathology
Cilia - ultrastructure
ciliary motion
ciliary ultrastructure
Female
Follow-Up Studies
Humans
Kartagener Syndrome - diagnosis
Male
Medical sciences
Microscopy, Electron
Nasal Mucosa - ultrastructure
Pneumology
Prevalence
primary ciliary dyskinesia
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Summary:The purpose of this study was to distinguish between acquired and genetically determined ciliary abnormalities in children with severe chronic respiratory diseases. Samples of nasal ciliated epithelium from 50 subjects (25 male, 25 female; age‐range 2–19 years) with severe chronic respiratory diseases were examined using transmission electron microscopy (TEM). Based on TEM findings, patients were divided into two groups: A and B. Group A comprised 39 children with ciliary alterations compatible with a condition probably occuring secondary to chronic inflammation (alterations of peripheral pairs, swollen cilia, and compound cilia). The other 11 patients, Group B, exhibited a greater number of alterations of the central pair and dynein arms (p
ISSN:0905-6157
1399-3038
DOI:10.1046/j.0905-6157.2001.00000.x