Vol 62, No 4 (2003)
Short communication
Submitted: 2012-02-06
Published online: 2003-09-05
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.
Vol 62, No 4 (2003)
SHORT COMMUNICATIONS
Submitted: 2012-02-06
Published online: 2003-09-05
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.
Keywords
immotile cilia syndrome; electron-microscopy
Title
Changes in the bronchial epithelia in patients with immotile cilia syndromes
Journal
Folia Morphologica
Issue
Vol 62, No 4 (2003)
Article type
Short communication
Pages
393-395
Published online
2003-09-05
Page views
631
Article views/downloads
869
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