What Is Kartagener's Syndrome?

Kartagener consists of a triad of bronchiectasis, chronic sinusitis or nasal polyps, and visceral paralysis. It is mainly characterized by recurrent respiratory suppurative infection, hemoptysis, bronchiectasis, paranasal sinusitis, and right heart. Kartagener, also known as visceral inverse-sinusitis-bronchiectasis syndrome, or familial bronchiectasis, is a congenital autosomal recessive disease with a family genetic predisposition, which can occur in the same or the same generation. Has a close marriage history.

Basic Information

nickname: Visceral inverse-sinusitis-bronchiectasis syndrome, familial bronchiectasis
English name: Kartagener syndrome
Visiting department: Internal medicine

Multiple groups: Patients before the age of 15
Common causes: May be recessive or dominant
Common symptoms: Repeated cough, bloody pus, phlegm, fever, dyspnea, cyanosis, etc.

Causes of Kartagener syndrome

The cause may be recessive or dominant, and some people do not think this disease is a genetic disease.

Kartagener syndrome clinical manifestations

Due to the disturbance of respiratory ciliary epithelium, the mucociliary transport function declines, and secretions cannot be discharged, which causes repeated long-term chronic infection, which forms the pathological basis of bronchiectasis and parasinusitis. Ciliates are widely found in the respiratory tract, middle ear, fallopian tubes, sperm flagella, and other organs such as the brain and spinal cord membranes. Kartagener syndrome can be accompanied by pneumonia, conductive deafness, ectopic pregnancy, infertility, and hydrocephalus. The age of first onset of this disease is often in childhood, but it is easy to be misdiagnosed. Repeated coughing, sputum, hemoptysis, stuffy nose, runny nose, dizziness, headache, etc. A few see a doctor for infertility.

Most often develop symptoms before the age of 15, and have a repeated cough since childhood, cough and sputum with bloodshot or hemoptysis, and fever, dyspnea, cyanosis, etc., lungs and wet murmurs. Patients have chronic rhinitis, paranasal sinusitis, or nasal polyps, with occasional melanoconjunctivitis. Total visceral translocation, and may be accompanied by other deformities, such as atrial septal defect, ventricular septal defect, hydrocephalus, cleft lip, deafness and so on.

Kartagener syndrome test

The total number of peripheral blood leukocytes can increase. Chest X-ray examination showed increased lung texture and cardiac translocation; bronchograms showed bronchiectasis, which were more common in the lower lobe of both lungs.

Kartagener syndrome diagnosis

The possibility of Kartagener should be considered in patients with bronchiectasis and visceral transposition. Imaging is an important basis for clinical diagnosis of Kartagener syndrome. Chest x-rays, CT scans, and ultrasound examinations can reveal visceral translocation.

Kartagener syndrome treatment

Antibiotics are mainly applied to prevent sinusitis or respiratory infections. Severe bronchiectasis, frequent hemoptysis or infection, and confined to one side of the lung may be considered surgical treatment. Immunomodulators can be used to administer influenza and / or pneumococcal vaccines to increase resistance and help reduce respiratory infections.

Principles of treating sinusitis in Kartagener syndrome:

Conservative treatment

Including acute sinus and lung diseases, select sensitive antibiotics after clearing the pathogen according to the secretion culture results; those with nasal polyps can use intranasal glucocorticoids; those with allergic rhinitis can use antihistamine ; Topical use of saline, antibiotics (aminoglycosides) or fungicide intranasal lavage. Surgical treatment is needed when conventional conservative treatment is ineffective, especially when nasal secretion cultures indicate drug-resistant bacteria or characteristic pathological changes, or with nasal polyps.

2. Principles of surgical treatment

Based on the resection of irreversible lesions, the morphology and physiological functions of the nasal cavity and sinus mucosa were improved by reconstructing the nasal cavity, sinus ventilation and drainage pathways. With the further understanding of the disease and the continuous development of nasal endoscopic surgery, nasal-sinus diseases of Kartagener syndrome are more advocated for more aggressive surgical treatment. Effective treatment may reduce the progress of bronchial and lung diseases.