Kartagener syndrome

Kartagener syndrome: Description, Causes and Risk Factors:Complete situs inversus associated with bronchiectasis and chronic sinusitis associated with ciliary dysmotility and impaired ciliary mucous transport in the respiratory epithelium; autosomal-recessive inheritance with variable penetrance. The mechanism of the reversal of laterality remains an enigma, but it appears to be strictly an abolition (indifference) of laterality rather than a true reversal.Kartagener syndromeIt is usually diagnosed in infancy or early childhood. It can occur in all races and is equally present in boys and girls. The incidence is thought to be approximately 1 in 40,000.Kartagener syndrome is a rare hereditary disease caused by a gene defect. Primary (genetic) defects in the structure and function of sensory and motile cilia result in multiple ciliopathies. A reduction in the number of arms which propel mucus (dynein arms) is a common abnormality but many other structural defects of the cilia have been found. Patients with normal cilia morphology but abnormal mucus propulsion have been detected. In diagnosing the syndrome, therefore, both ciliary structure and motility need to be assessed.It consists of a triad of features:Situs inversus (transposition of the viscera).
  • Abnormal frontal sinuses (producing sinusitis and bronchiectasis).
  • Primary ciliary dyskinesia (PCD).
The defective cilia lining the respiratory tract are unable to clear the airways of secretions and pathogenic bacteria, resulting in mucus retention and chronic or recurrent respiratory tract infection - leading to damage to airway walls.Symptoms:Most of the symptoms of Kartagener syndrome result from the inability of the respiratory cilia to function correctly, such as:Chronic sinus infection.
  • Frequent lung infections, such as pneumonia and bronchitis.
  • Bronchiectasis - lung damage from frequent infections.
  • Frequent ear infections.
  • The important symptom that distinguishes Kartagener syndrome from other types of primary ciliary dyskinesia is positioning of the internal organs on the side opposite from normal (called situs inversus).
Diagnosis:Kartagener syndrome is recognized by the three main symptoms of chronic sinusitis, bronchiectasis, and situs inversus. Chest x-ray or computed tomography (CT) scan can detect lung changes characteristic of the syndrome. Taking a sample (biopsy) of the lining of the trachea, lung, or sinuses can allow microscopic examination of the cells that line the respiratory tract, which can identify defective cilia.Treatment:Medical care for a person with Kartagener syndrome focuses on prevention of respiratory infections, and prompt treatment of any that may occur. Antibiotics can relieve sinusitis, and inhaled medications and respiratory therapy can help if chronic lung disease develops. Small tubes may be placed through the eardrums to allow infections and fluid to drain out of the middle ear. Adults, especially men, may have difficulty with fertility, and may benefit from consulting a fertility specialist.In many individuals, the number of respiratory infections begins to decrease by about age 20, and as a result many people with Kartagener syndrome have near normal adult lives.NOTE: The above information is educational purpose. The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition.DISCLAIMER: This information should not substitute for seeking responsible, professional medical care.

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