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Our body contains long string of dough called as glycosaminoglycans ( GAGs ) . These GAGs must be broken down so that they can be used for development of bones , connective tissues , etc . and they are commonly broken down by an enzyme name iduronate-2 - sulfatase ( I2S ) .
In Hunter syndrome , there is lack or absence of this enzyme I2S. Because of this the jape are not broken down and finally they start hoard in the cubicle and tissue . right ontogeny of bones and tissues is hampered due to lack of jape . Hence , Hunter syndrome is a familial disorder that shows multisystem interest .
How To Prevent Hunter Syndrome?
Hunter syndrome can be prevented with the help of PGD ( Pre - implantation Genetic Diagnosis ) . PGD is a procedure that is used before the implantation of fertilized egg . This method is very utile for foreclose Hunter syndrome.(1 )
Steps Involved In PGD-
Advantages Of PGD-
Disadvantages Of PGD-
This procedure can notice the inherited disorder before implantation but in some cases the upset may be undiagnosed , so even tests are to be done during pregnancy . Sometimes the patient role may show the symptom at the center age and hence it may stay undiagnosed .
There is no way to preclude Hunter syndrome once the mother has given birth . If the first child is diagnose with Hunter syndrome then the couple must look up a genetic pleader before planning for 2nd gestation . If the father is already diagnosed then preventive measure must be taken before having first child .
Complications Of Hunter Syndrome
Cardiovascular Complications – wrong line of descent supply to the electric organ due to thickening of heart tissue and this get improper functioning of the valve of heart . Gradually the heart can not function leading to heart failure . The patient also suffers from high blood pressure due to thickening of major blood vessels like aorta .
Respiratory Complications – Breathing difficulty due to macroglossia , thickened nasal passage and gum . Respiratory tract infections and pneumonia are very vulgar . Due to obstructed airways , the patient experience sleep apnea . Sleep apnea refer to a condition due to which there is intermittent disturbance of breathing during sleep .
Nervous System Complications – There is thickening of the membranes that cover the spinal electric cord and this leads to scarring . Due to this there is spinal cord compression . This answer in fatigue and helplessness of the lower limbs . When the disease is more severe , the patient show behavioral mental defectiveness . Children may regain difficultness centralise . Seizures are also common in patient role with Hunter syndrome .
Skeletal Complications – Due to accretion of gag in the connective tissue paper there is problem in right growth of bones causing stunted development of child . forcible miscreation are also common . Irregular shape of vertebra , arms , rib , etc . is seen . All these abnormalities related to bones in Hunter syndrome are collectively call in as dysostosis multiplex .
Conclusion
hunting watch syndrome or mucopolysaccharidosis type II can be prevented with the help of a diagnostic technique send for Pre - nidation Genetic Diagnosis ( PGD ) . This is a particular mental test where the embryo is prepared in vitro first . Then few cell from the embryo are collected and testes for the mien unnatural chromosome are done . This is the right method to prevent Hunter syndrome .
References-
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