Chest ImagingCiliary dyskinesia syndrome
(also called immotile cilia syndrome), disorder characterized by abnormal ciliary structure and function throughout the body. Males are infertile. The impaired mucociliary clearance is associated with recurrent upper and lower respiratory tract infections. In ciliary dyskinesia syndrome, there is an autosomal recessive pattern inheritance but there is genetic heterogeneity and discordance in phenotypic presentation. Bronchiectasis develops in childhood and adolescence and is associated with recurrent pneumonias. Radiological findings include
bronchial wall thickening,
bronchiectasis,
overinflation and
focal areas of
consolidation and
atelectasis. A subset of ciliary dyskinesia syndrome is
Kartageners syndrome (
Fig.1), which features situs inversus,
paranasal sinusitis and
bronchiectasis.
PG - MB
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a. Chest radiograph in a patient with Kartagener's syndrome showing situs inversus, diffuse overinflation and bilateral rounded opacities in lung bases.
b. HRCT scan showing bilateral bronchiectasis. The dilated bronchi in the lower lobes are filled with mucus.
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Ciliary dyskinesia syndrome, Fig.1 (a) | | Ciliary dyskinesia syndrome, Fig.1 (b) | |