What is Kartagener Syndrome?
Kartagener ’s Syndrome is an exceedingly rarefied inherit medical condition which is triggered by variation of different genes . It is an autosomal recessive trait mean that a shaver want to inherit two copies of the defective factor one from each parent to develop Kartagener ’s Syndrome . enquiry suggests that 1 in approximately 30,000 births develop Kartagener ’s Syndrome .
Kartagener ’s Syndrome causes defect in the respiratory tract but the most alarming feature article of this status is that it causes the position of many vital organs of the consistence to get reversed . This means that the heart which is on the left side of the body is located on the right side in a child with Kartagener ’s Syndrome . This phenomenon is termed as Situs Inversus .
As of now , there is no remedy for Kartagener ’s Syndrome and treatments are principally aim at managing the symptoms triggered by it and better the overall quality of life of the affected somebody .
How is Kartagener Syndrome Caused?
Different factor mutant ensue in a child to be suffer with Kartagener Syndrome . The function of these genes is to develop protein that control the functioning of cilia which are small hair like structures found in various parts of the body , peculiarly the respiratory system of rules .
A normal work cilia is super vital for normal functioning of many vital organ of the body . Mutations of these genes result in the cilia to become immobile resulting in the growth of Kartagener ’s Syndrome .
What are the Symptoms of Kartagener Syndrome?
There are two primary characteristic of Kartagener ’s Syndrome which are Primary Ciliary Dyskinesia and Situs Inversus . These conditions can cause variety of symptoms to let in perennial respiratory and venous sinus infections , recurrent ear infections , persistent adenoidal over-crowding , and even sterility in some citizenry by and by on in life story . These symptoms are mainly induce by Primary Ciliary Dyskinesia as Situs Inversus does not make any symptom .
How is Kartagener Syndrome Diagnosed?
Some case of Kartagener ’s Syndrome are diagnosed as early as at the prison term of nativity of the child . The nestling will show respiratory suffering and will require oxygen for respiration . However , maximum cases are identified during childhood when the child experiences frequent repeated venous sinus and respiratory infection . The child will also suffer from frequent infections of the ears as well . The investigating conduct to identify the cause of these conditions will not be conclusive . This will make the address physician suspect Kartagener ’s Syndrome .
To confirm the diagnosis the Dr. will perform trial to attend for presence of nasal polyps . A nipper with Kartagener ’s Syndrome will have a diminish sense of smell which will also be tested while diagnosing Kartagener ’s Syndrome . A hearing test will be done to check for any sign of hearing going which also is a feature of Kartagener ’s Syndrome .
Additionally , imagination studies , tissue biopsies , and genetical tests will be done to confirm the diagnosis of Kartagener ’s Syndrome . A breathing trial often is done to support the diagnosis of Kartagener ’s Syndrome . A fry with this condition tends to breathe out less nitric oxide than normal . Thus a breathing exam along with the tests mentioned above will confirm the diagnosing of Kartagener ’s Syndrome .
How is Kartagener Syndrome Treated?
Kartagener ’s Syndrome does not have any cure . The management for this shape is aim at carry off the symptom and preventing any complications that may arise due to Kartagener ’s Syndrome . The patient may be prescribed downcast dose antibiotics to handle the frequent respiratory and fistula infections . For spartan transmission , steroids may be used in limited amount .
To drain out the collection of mucose from the lungs , a bronchoscopy may have to be done . In uttermost case where the lungs get severely damage then a transplantation may be required but such instances are quite rare .
Majority of people with Kartagener ’s Syndrome lead a normal life without any problems provided they cling to the good word of their treat physician and go for unconstipated followups to prevent complication .
