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What is Hunter Syndrome?
Hunter Syndrome is an exceedingly rare inherited medical stipulation characterize by a dysfunctional enzyme call iduronate 2 - sulfatase.(1)This enzyme functions by breaking down certain extremely complex molecular structures in the body and aid in liquid functioning of the organs of the body . A depletion of unadulterated absence of this enzyme cause buildup of these molecular structures which is extremely harmful for the body.(1 )
Gradual buildup of these molecular structures results in permanent legal injury to various parts of the dead body , which affect the overall mental growth of the patient.(1)Hunter Syndrome also ensue in dysfunction of various reed organ system . The forcible social organisation of the patient also gets affected as a result of Hunter Syndrome . Hunter Syndrome is more often pick up in son than young woman .
Hunter Syndrome belongs to mucopolysaccharidosis , which is a group of inherit metabolic conditions.(2)Hunter Syndrome does not have a therapeutic and treatment is aimed at controlling symptom and preventing any complications arise out of this condition.(3 , 4)Hunter Syndrome is an ten - linked recessive upset in which the defective cistron is inherited from one parent.(5 )
Hunter Syndrome is a progressive term and the overall aesculapian condition of the affected person bit by bit worsens with meter . This article details some of the presenting features of Hunter Syndrome .
What are the Presenting Features of Hunter Syndrome?
The presenting symptom of Hunter Syndrome are significantly varying depending on the extent of the disease and ranges from mild to severe . The presenting feature of Hunter Syndrome may not be patent instantly after the birth of a shaver , but by and large spring up by the clock time the tike is around three to four years of age.(6)This is when there is build - up of the molecular structure due to the nonadaptive enzyme bear on functioning of various vital organs of the body .
A child with Hunter Syndrome will have an exaggerated head.(8)The lips of the patient suffering from Hunter Syndrome will also be abnormally thick.(8)The nose of the affected role have Hunter Syndrome will also be broad with a bulge tongue.(7 , 8)The voice will also be hoarse along with other musculoskeletal abnormalities with abnormally shaped bone in Hunter Syndrome.(8)The internal organs of patient with Hunter Syndrome also get enlarged as a result of which the abdomen becomes distended .
There are also certain dermatological abnormality see in people with Hunter Syndrome with white colored blister like cutis growths.(8)Behavior Stephen Samuel Wise , people with Hunter Syndrome have abnormally aggressive behavior .
There are also developmental delays in patients suffering from Hunter Syndrome . The development ; however , remains normal for the first two eld of life of the patient and when the symptoms first start to appear , then the growth retardation begins . Joint stiffnessand frequent episode ofdiarrheais also quite common in people with Hunter Syndrome .
Hunter Syndrome is extremely rare ; however , if a fry read significant change in facial appearance , growth retardation , and musculoskeletal freakishness among other symptoms seen with this condition , then it is best to look up with a physician to rule in or out Hunter Syndrome .
Conclusion
In conclusion , Hunter Syndrome is a rarified inherited precondition in which the enzyme that breaks down complex molecular social structure for smooth functioning of the consistence . Due to malfunction of this enzyme there is accumulation of these molecular structures , which bit by bit get going affecting the operation of vital organs of the body . There is no remedy for Hunter Syndrome .
The presenting features of Hunter Syndrome are not seeable till the fry is about two year of age . This is when parents start comment significant forcible and behavioral changes in the child . Musculoskeletal mental defectiveness , such as unnatural shape and size of it of the castanets are also quite common in Hunter Syndrome .
Thus , if a shaver is observed to have any of the symptom mentioned above then you should consult with a physician for an accurate diagnosing so that a handling architectural plan is devised to best suit the patient and prevent complications from Hunter Syndrome .
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