Kartagener ’s syndrome is also called as Primary cilial dyskinesia ( PCD ) or Immotile ciliary syndrome . It is a rare hereditary syndrome and affects the airways . Till appointment there is no known therapeutic for Kartagener ’s syndrome and the intervention is only by the management of its symptoms .
What is Kartagener’s Syndrome?
Primary ciliary dyskinesiais caused due to defects in the cilia lining of the respiratory parcel , fallopian tubes and flagella of the sperm . Cilia are bantam hair like structures and their chief function is to move mucous secretion out of the respiratory passing or attend to is the movement of ovum in the fallopian tube or sperm in the scourge . During infection more mucus is formed and due to their inefficient movement , unnatural cilia can not remove this mucous secretion out through the nozzle and throat . Thus , the mucus becomes thick and block the respiratory tract lead to various knottiness .
When master ciliary dyskinesia is go with by situs inversus , chronic sinusitis and bronchiectasis , it is call as Kartagener ’s syndrome ( KS ) . Situs inversus mean all of the vital organ in the chest andabdomenregion are placed in reversed club as compare to their normal position . For illustration , liver , which is usually on the right , gets place on the left and still it performs its subprogram in a normal fashion .
Kartegener ’s syndrome pass in 50 % of the primary ciliary dyskinesia causa . It is a rarefied autosomal recessive upset .
Symptoms of Kartagener’s Syndrome
Due to impaired move of cilia , there is trouble in the headroom of mucous secretion from thelungs , which increases the peril of infections .
Primary ciliary dyskinesia have a number of symptom as :
Some cause of primary ciliary dyskinesia exhibit chronic bronchiectasis , chronic sinusitis andotitis media .
Although there is switch of some or all vital organs of the thorax and abdomen , situs inversus does not cause much wellness problems . The heart and other vital organ probably function in a normal manner .
Prevalence Rate of Kartagener’s Syndrome
The frequence of Kartagener ’s syndrome is estimated to be 1 in 30,000 live birth . There is no predilection for sex . It mostly occurs in puerility stage and settle in adulthood .
Prognosis of Kartagener’s Syndrome
It is observed that patient role with kartagener ’s syndrome have unmanageable childhood due to lasting infections involve the respiratory tract , sinus and ears . It severely affects the first decade of biography but ameliorate in the adulthood .
Chronic infections if not treated in time , direct to severe clinical symptom with risk of infection of minify pulmonary function . In such case , if bilateral lung organ transplant is hold up the prognosis can be liveliness - ominous .
Causes of Kartagener’s Syndrome
Kartagener ’s syndrome is induce due to the ciliate defect , which is an inherited familial defect . More than 200 genes are entail to play a role in primary ciliary dyskinesia . The reduction in the number of cilia which propel mucus is a very rough-cut job although a identification number of other structural abnormalities are present .
Pathophysiology of Kartagener’s Syndrome
Patients with main cilial dyskinesia show a wide regalia of defects in ultrastructure of the cilia and its motion . The defect in cilia motility impairs cilial beating function and impairs the mucous secretion clearing power . The vernacular defect detect is reduction of dynein arms , which minify the ciliary beat frequency and cilium do not go around .
Additionally , there is a probability of some chemical nitty-gritty , which interfere with normal performance of cilia .
Risk Factors of Kartagener’s Syndrome
Complications of Kartagener’s Syndrome
Some ramification of kartagener ’s syndrome are :
Diagnosis of Kartagener’s Syndrome
The patient may have only symptoms of kartagener ’s syndrome and not show situs inversus .
At times kartagener ’s syndrome is name in newborns who exhibit respiratory distraint and demand atomic number 8 therapy . It is commonly diagnosed during puerility .
diagnosing of kartagener ’s syndrome include checking for signs of :
Other trial run let in :
Treatment of Kartagener’s Syndrome
Till escort there is no known cure for kartagener ’s syndrome . However , a medico can dictate a treatment plan , which can aid in the direction of the symptoms and decrease risk of complication .
By following the doctor ’s advice and biannual follow up visits , many patients with kartagener ’s syndrome lead a normal .
Kartegener ’s syndrome is a rare hereditary upset , caused due to defects in the cilia . There is still no cure for it . Current enquiry activities are increasing the knowledge regarding pathogenesis of this syndrome . This effort , in time to come will lead to respectable therapeutics point at improving life of kartagener ’s syndrome patients .
