Farnaz Gheisari,1Saman Hakimian,2,*
2. M.sc student of Pathogenic Microbes Islamic Azad University Central Tehran Branch
Introduction: Kartagener's syndrome is described as autosomal recessive inherited syndrome.
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.
Methods: Patients with normal cilia morphology but abnormal mucus propulsion have been detected. Symptoms of kartagener :
inversion of the circulatory system and the viscera. This is an incorrect placement of the organs on the opposite side from the corresponding one.
swelling of the sinuses, that is, the spaces inside the nose and head.
dilation of the bronchi, which can cause asthma in patients.
The important symptom that distinguishes Kartagener syndrome from other types of primary ciliary dyskinesia is the positioning of the internal organs on the side opposite from normal (called situs inversus). For example, the heart is on the right side of the chest instead of the left. The main symptom of Kartagener syndrome is lung problems. Because the cilia don’t work properly, you have trouble moving debris and fluid from your lungs. The severity of symptoms can vary for each person, but they usually start at birth. Primary ciliary dyskinesia is a genetic condition where the cilia aren't working properly.
Results: Cilia are hair-like structures on the surface of your cells and are found in your lungs, airways, and other areas. They move in a wave-like motion to help position organs during a baby's growth in the womb, and they help move out mucus, bacteria, and debris from your lungs and airways.
(PCD) also known as Kartagener's syndrome can present with cough, multiple pneumonia, bronchiectasis, rhinosinusitis, infertility, among others, but it can rarely present with massive. In PCD and Kartagener syndrome, these cilia don't move or they don't move very well, which causes ongoing lung, sinus, and ear problems. Not all people with PCD have reversed organs, so Kartagener syndrome is a subtype of the disease. Disorders of ciliary motility may be congenital or acquired. Congenital disorders are labeled as PCDs. Nearly 50% of PCD patients have situs inversus. Such cases of PCD with situs inversus are known as Kartagener's syndrome. Male patients with KS invariably present infertility, while women present reduced fertility.Infertility in male KS patients is due to diminished sperm motility, while in females it is due to defective ovum transport because of dyskinetic motion of oviductal cilia, suggesting that the ciliated endosalpinx is essential for human reproduction.
Conclusion: Normally, the diagnosis of Kartagener's Syndrome is complicated because it is a rare disease and each person shows some symptoms. There is no cure for Kartagener syndrome. The main treatments focus on keeping airways clear and loosening
and getting rid of thick, extra fluid, mucus, and
debris. It's also important to get vaccines to prevent serious disease from viruses.