Kartagener syndrome is listed as a "rare disease" by the Office of Rare Diseases (ORD) of the National Institutes of Health (NIH). This means that Kartagener syndrome, or a subtype of Kartagener syndrome, affects less than 200,000 people in the US population.
Ophanet, who are a consortium of European partners, currently defines a condition rare when if affects 1 person per 2,000. They list Kartagener syndrome as a "rare disease". More information about Kartagener syndrome is available from Orphanet
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Because of the availability of antibiotics to treat acute respiratory tract infections, the incidence of bronchiectasis has dramatically decreased in the past 20 years. Incidence is highest among Eskimos and the Maoris of New Zealand. It affects people of both sexes and all ages.
The different forms of bronchiectasis may occur separately or simultaneously. In cylindrical bronchiectasis, the bronchi expand unevenly, with little change in diameter, and end suddenly in a squared-off fashion. In varicose bronchiectasis, abnormal, irregular dilation and narrowing of the bronchi give the appearance of varicose veins. In saccular bronchiectasis, many large dilations end in sacs. These sacs balloon into pus-filled cavities as they approach the periphery and are then called saccules. (See Forms of bronchial dilatation, page 560.)
This disease results from conditions associated with repeated damage to bronchial walls and abnormal mucociliary clearance, which cause a breakdown of supporting tissue adjacent to airways. Such conditions include:
❑ cystic fibrosis
❑ immunologic disorders (agammaglobulinemia, for example)
❑ recurrent, inadequately treated bacterial respiratory tract infections, such as tuberculosis, and complications of measles, pneumonia, pertussis, or influenza
❑ obstruction (by a foreign body — most common in children, tumor, or stenosis) in association with recurrent infection
❑ inhalation of corrosive gas or repeated aspiration of gastric juices into the lungs
❑ congenital anomalies (uncommon), such as bronchomalacia, congenital bronchiectasis, immotile cilia syndrome, and Kartagener’s syndrome, a variant of immotile cilia syndrome characterized by situs inversus, bronchiectasis, and either nasal polyps or sinusitis.
In bronchiectasis, hyperplastic squamous epithelium denuded of cilia replaces ulcerated columnar epithelium. Abscess formation involving all layers of the bronchial wall produces inflammatory cells and fibrous tissue, resulting in dilation and narrowing of the airways. Mucus plugs or fibrous tissue obliterates smaller bronchioles, whereas peribronchial lymphoid tissue becomes hyperplastic. Extensive vascular proliferation of bronchial circulation occurs and produces frequent hemoptysis.
Source: Professional Guide to Diseases (Eighth Edition), 2005
The term 'prevalence' of Kartagener syndrome usually refers to the estimated population of people who are managing Kartagener syndrome at any given time. The term 'incidence' of Kartagener syndrome refers to the annual diagnosis rate, or the number of new cases of Kartagener syndrome diagnosed each year. Hence, these two statistics types can differ: a short-lived disease like flu can have high annual incidence but low prevalence, but a life-long disease like diabetes has a low annual incidence but high prevalence. For more information see about prevalence and incidence statistics.
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