Kartagener Syndrome
Kartagener syndrome is an inherited disorder characterized by bronchiectasis, chronic sinusitis and otitis, and situs inversus, or transposition of the internal organs.
In Kartagener syndrome, as in primary ciliary dyskinesia, an inherited defect results in ciliary dysfunction throughout the pulmonary and sinonasal passages. The result is impaired clearance, pooling of secretions in the tracheobronchial tree, and irritation of the airways by trapped particles and bacteria. Additional secretion production results as the natural defense mechanisms of the lung attempt to clear these invading particles and organisms, thus exacerbating the problem of secretion retention.
Bronchiectasis, a diagnostic feature of Kartagener syndrome characterized by an abnormal widening of the bronchi and mucus hyperproduction, contributes even more to secretion retention.
Retained secretions provide a cultural medium for bacterial pathogens. Consequent infections produce still more mucus, perpetuating a vicious cycle of mucus obstruction, recurrent bouts with pneumonia, bacterial colonization, progressive pulmonary compromise and, finally, respiratory failure.
In addition to an increased risk of pulmonary infection, secretion retention may result in mucus plugging and impaired gas exchange, pulmonary hyperinflation, thickening of the bronchial walls, and atelectasis.
Treatment strategies for Kartagener syndrome include aggressive, daily airway clearance therapy. Such therapy is recognized as an essential component in managing and minimizing the obstruction, infection, inflammation and subsequent pulmonary dysfunction which contribute significantly to medical care costs, morbidity, and mortality in afflicted individuals.
Sources
Teknos TN, Metson R, Chasse T, Balercia G, Dickersin GR. New developments in the diagnosis of Kartagener syndrome. Otolaryngol Head Neck Surg 1997; 116 (1): 68-74. Dombi VH, Walt H. Primary ciliary dyskinesia, immotile cilia syndrome, and Kartagener syndrome: diagnostic criteria. Schweiz Med Wochenschr 1996; 126 (11): 421-433.
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