open access

Vol 62, No 4 (2003)
Short Communications
Published online: 2003-09-05
Submitted: 2012-02-06
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Changes in the bronchial epithelia in patients with immotile cilia syndromes

Wiesława Biczysko, Andrzej Marszałek, Monika Seget, Anna Bręborowicz, Joanna Goździk, Grażyna Sierakowska-Urbańska
Folia Morphol 2003;62(4):393-395.

open access

Vol 62, No 4 (2003)
Short Communications
Published online: 2003-09-05
Submitted: 2012-02-06

Abstract

Immotile cilia syndromes is a cause of recurrent infection of the airways and recurrent bronchopneumonias. Among the ciliary abnormalities are found changes in the structure of the microtubules, unco-ordinated ciliary movements caused by the absence of inner or outer or both dynein arms, and abnormalities of the kinetosomes and/or rete ridges. In patients with ciliary dyskinaesia bronchitis occurs early in life (during infancy) and usually has a recurrent tendency, so that bronchial biopsy is frequently undergone for diagnostic purposes. In this study we include 127 bronchial biopsies from patients (from 2 months to 49 years) unsuccessfully treated for recurrent respiratory tract infections. When performing regular diagnostic procedures on the light and electron microscopic level, we have looked for cilia abnormalities and also focused on changes within the mucosa and submucosa. The most common abnormality recorded was absence of the inner dynein arms, but in 40 cases neither of the dynein arms were present. Only a few patients had classical Kartagener’s syndrome. Special attention is drawn to biopsies from elderly patients, in whom long-standing infections were followed by extensive damage to the bronchial epithelium, including even a total absence of ciliated cells. In some cases enhanced regenerative processes and some foci of squamous metaplasia were found. In two cases even foci of low-grade dysplasia were diagnosed.

Abstract

Immotile cilia syndromes is a cause of recurrent infection of the airways and recurrent bronchopneumonias. Among the ciliary abnormalities are found changes in the structure of the microtubules, unco-ordinated ciliary movements caused by the absence of inner or outer or both dynein arms, and abnormalities of the kinetosomes and/or rete ridges. In patients with ciliary dyskinaesia bronchitis occurs early in life (during infancy) and usually has a recurrent tendency, so that bronchial biopsy is frequently undergone for diagnostic purposes. In this study we include 127 bronchial biopsies from patients (from 2 months to 49 years) unsuccessfully treated for recurrent respiratory tract infections. When performing regular diagnostic procedures on the light and electron microscopic level, we have looked for cilia abnormalities and also focused on changes within the mucosa and submucosa. The most common abnormality recorded was absence of the inner dynein arms, but in 40 cases neither of the dynein arms were present. Only a few patients had classical Kartagener’s syndrome. Special attention is drawn to biopsies from elderly patients, in whom long-standing infections were followed by extensive damage to the bronchial epithelium, including even a total absence of ciliated cells. In some cases enhanced regenerative processes and some foci of squamous metaplasia were found. In two cases even foci of low-grade dysplasia were diagnosed.
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Keywords

immotile cilia syndrome; electron-microscopy

About this article
Title

Changes in the bronchial epithelia in patients with immotile cilia syndromes

Journal

Folia Morphologica

Issue

Vol 62, No 4 (2003)

Pages

393-395

Published online

2003-09-05

Bibliographic record

Folia Morphol 2003;62(4):393-395.

Keywords

immotile cilia syndrome
electron-microscopy

Authors

Wiesława Biczysko
Andrzej Marszałek
Monika Seget
Anna Bręborowicz
Joanna Goździk
Grażyna Sierakowska-Urbańska

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