Kartagener Syndrome

Kartagener Syndrome Kartagener syndrome is a chronic lung disease whose special feature is the incapacity of cilia to move. Kartagener syndrome occurs very rarely, 1-32 000 cases, is a birth defect and affects men and women equally. Individuals with Kartagener syndrome shows abnormal motility of the cilia, which causes a specific clinical picture, dominated by respiratory infections. Diagnosing the disease is made by imaging investigations and treatment target antibiotic prophylaxis and infectious processes. Kartagener syndrome is also called: primary ciliary dyskinesia, immobile cilia syndrome, Afselius syndrome, Kartagener Triad, Zivert's syndrome and Zivert-Kartagener triad.
Etiology Kartagener syndrome is caused by ciliary defects. Most patients are born with the disease.
What are cilia? Cilia are hair-like structures present on the surface of many cell types. One function of cilia is to increase the elimination of mucus from the respiratory tract. Infections cause excess mucus production and cilia are immobile due to their inefficient or motility, it blocks the respiratory tract, causing various complications. Cilia are hair-like structures present on the surface of many cell types. One function is to promote cilia. Infections cause excess mucus production and cilia are immobile due to their inefficient or motility, it blocks the respiratory tract, causing various complications.
Clinical
Most Kartagener syndrome symptoms that occur from respiratory cilia inability to function properly. The clinical picture includes: - Chronic sinus infections; - Frequent lung infections: pneumonia, bronchitis; - Bronchiectasis: pulmonary damage due to repeated infections; - Frequent ear infections; Kartagener syndrome main symptom apart from other types of primary ciliary dyskinesia is reverse positioning of internal organs (situs inversus). For example, instead of being on the left side of the chest, heart on the right side.
Diagnosis
Kartagener syndrome diagnosis is based on symptomatic triad: chronic sinusitis, and situs inversus bronsiectazie. With chest X-ray or pulmonary computertomografiei can detect the characteristic changes that occur in this syndrome. Biopsy a sample of tissue from the lining of the trachea, lung and sinus allows microscopic examination of cells lining the respiratory tract and identify defective cilia.
Treatment
Medical care is given to a person with Kartagener syndrome focuses on prevention of respiratory infections and prompt treatment of them. In case of sinusitis is adminsitreaza antibiotics, and in case of chronic lung diseases may be helpful for inhaled medications. To increase the elimination of infections and middle ear fluid, the doctor puts some small devices in the ear canal, the tubular shape. Adults, especially men, may have fertility troubles, so we recommend consulting a specialist. In most cases, the number of respiratory Since infections begins to decrease after age 20 years, so many patients with Kartagener syndrome lead a normal life.
Complications
Complications of Kartagener syndrome are: - Bronchiectasis. : Occurs when arborlui bronchial branches are blocked and chronically infected, causing weakening and dilation of the branches. Dilated tubes allow the accumulation of mucus and bacteria, leading to infection and pneumonia; - Dyspnea (difficulty breathing); - Chronic wheezing, cough;