Primary Ciliary Dyskinesia Associated with Kartagener's Syndrome

Primary ciliary dyskinesia is a genetic disorder of abnormal ciliary structure and function that leads to defective mucociliary clearance, resulting in sinopulmonary infection and infertility. Kartagener's syndrome is a subclass of primary ciliary dyskinesia and which is clinically distinguishable by the presence of situs inversus. Electron microscopy of cilia reveals ultrastructural defects in the ciliary axoneme. In the respiratory tract, this inability presumably causes impaired clearance of mucus and inhaled particles and results in the chronic infections of sinuses and bronchial trees that are characterized of the disease. Early diagnosis of the disease may be important for prevention of bronchiectasis and pulmonary insufficiency.

We report one case of primary ciliary dyskinesia associated with Kartagener's syndrome in a 14 year-old young man who was presented as bronchiectasis, chronic and situs inversus, with review of literature.