Primary ciliary dyskinesia (Kartegner syndrome)
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Cough in patient with known history of chronic lung disease.
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Cylindrical and varicose bronchiectatic changes seen predominantly in the right middle and lower lung lobes and the lingula. The upper lobes are relatively spared.
Diffuse bilateral centrilobular nodular changes with tree-in-bud configuration seen predominantly in the middle and lower lung lobes bilaterally.
The cardiac apex is seen pointed to the right side indicating dextrocardia.
The visible abdomen confirms situs inversus totalis.
The characteristic lung findings of cylindrical and varicose bronchiectasis with tree-in-bud configuration in the lingula, middle and lower lung lobes along with dextrocardia make primary ciliary dyskinesia the most likely diagnosis.
Note the relative sparing of the upper lobes.
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