Kartagener syndrome is a rare, ciliopathic, autosomal recessive genetic disorder that causes a defect in the action of the cilia lining the respiratory tract and fallopian tube. Kartagener syndrome definition of kartagener syndrome by. Cilia are tiny, hairlike structures that are found on the surface of cells in various parts of the body such as the lining of the airway, the reproductive system, and other organs. Learn about its symptoms and how its diagnosed and treated. Chest xrays or computed tomography ct scans can detect lung changes characteristic of the syndrome. Kartagener syndrome is recognized by the three main symptoms of chronic sinusitis, bronchiectasis and situs inversus. Kartageners syndrome is a rare genetic disease that causes problems with your airways. Patients usually present with chronic recurrent rhinosinusitis, otitis media, pneumonia, and bronchiectasis caused by pseudomonal infection. These genes encode proteins that are important to the structure and function of cilia. Taking a biopsy of the lining of the trachea, lung or sinuses can allow microscopic examination of the cells that line the. Situs inversus, bronchiectasis, and sinusitis and its relation to immotile cilia.
The mechanism of the reversal of laterality remains an enigma, but it appears to be strictly an abolition. Kartagener syndrome can be caused by changes mutations in many different genes. An autosomal recessive disorder characterized by a triad of dextrocardia. Kartagener syndrome definition and patient education. Approximately 50% of patient with primary ciliary dyskinesia.
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