title: "Cyst-like" structures within the ciliary shafts in children with bronchiectasis.
abstract: "Cyst-like" structures within the ciliary shafts were considered in four adults as a primary defect involved in the development of bronchiectasis. In this study, the presence and the primary or secondary nature of this abnormality were assessed in children with bronchiectasis. High resolution computed tomography (HRCT) and nasal biopsies for motion analysis and transmission electron microscopy (TEM) evaluation of cilia were obtained in 45 children with recurrent lower airway infections and abnormal chest radiography. HRCT disclosed bronchiectasis in 35 out of 45 (77.8%) children and cyst-like structures were demonstrated with TEM in 29 out of 45 (64.4%) patients. Cyst-like structures were constantly associated with other ultrastructural abnormalities commonly observed in chronic inflammation, and were found both in subjects with primary and with secondary ciliary dyskinesia. When considering only patients with bronchiectasis, a significant correlation between prevalence of cyst-like structures and the severity of bronchiectasis was demonstrated. Follow-up (2-22 months) of seven patients demonstrated that in the five children with secondary dyskinesia, the ultrastructural defect completely disappeared and there was a small reduction in the abnormality in the two patients with primary dyskinesia. In contrast to one previous report, the reversibility of the defect suggests its secondary origin, which is most likely related to chronic airway inflammation.
authors.affiliation: Dept of Pediatrics, University of Pisa, Pisa, Italy. m.pifferi@med.unipi.it
authors.forename: M
authors.initials: M
authors.lastname: Pifferi
authors.forename: A M
authors.initials: AM
authors.lastname: Cangiotti
authors.forename: D
authors.initials: D
authors.lastname: Caramella
authors.forename: A
authors.initials: A
authors.lastname: Pietrobelli
authors.forename: V
authors.initials: V
authors.lastname: Ragazzo
authors.forename: E
authors.initials: E
authors.lastname: De Marco
authors.forename: P
authors.initials: P
authors.lastname: Macchia
authors.forename: S
authors.initials: S
authors.lastname: Cinti
authors.forename: A L
authors.initials: AL
authors.lastname: Boner
mesh_terms: D000293:Adolescent; D001706:Biopsy; D001987:Bronchiectasis / Q000000981:diagnostic imaging / Q000473:pathology*; D002648:Child; D002675:Child, Preschool; D002923:Cilia / Q000648:ultrastructure; D003560:Cysts; D005260:Female; D006801:Humans; D007223:Infant; D008297:Male; D008854:Microscopy, Electron; D014057:Tomography, X-Ray Computed