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Orion syndrome is an X linked recessionary genetic disorderliness and is also known as mucopolysaccharidosis case II ( MPS II ) . It is a disease with multisystem involvement . There are long chain of shekels and carbohydrate in the cell of our body called glycosaminoglycans ( GAG ) that must be reveal down . An enzyme named iduronate-2 - sulfatase does the work of breaking down these glycosaminoglycans . But in hunter syndrome , there is deficiency of this enzyme and hence the GAGs build up up and accumulate in the cells and tissues and cause fuss with normal operation of the body . These GAGs are very crucial for the development of hide , sinew , cartilage , bone , etc . and hence they need to be broken down for its functioning .
How Often Does Hunter Syndrome Occur?
hunting watch syndrome is an X - yoke recessive genetic upset that is qualify by accumulation of the glycosaminoglycans in the body .
ordinarily the female parent has two X chromosomes and the forefather has one X chromosome and one wye chromosome . The mother inherits the X chromosome with the abnormal gene on it . The female person are not impress because of healthy cristal chromosome that is inherited from the father ; they are just the attack aircraft carrier of the abnormal gene . Males are affected always because they inherit the X chromosome from mother , which has the abnormal gene and other Y chromosome is inherited from the Father-God that is normal .
Females are the carriers and do not show any symptoms because only one X chromosome is abnormal and the other ex chromosome is normal.(1 ) ( 2 )
But all the sons of such mothers are not regard . There are 25 % hazard that the mother will give birth to a son with this disease and 25 % chances that she will give birth to a normal unaffected son . Also there are 25 % luck that the daughter of a bearer mother will transfer the toter chromosome to the girl and 25 % chances that she will give birth to a non - carrier daughter .
If a male has this disease , he will pass his abnormal X chromosome to all his daughters who will then become toter but his sons will not be affected because the begetter fall out on the Y chromosome and not the 10 chromosome . But in some cases , female can also be affected because of inactivation of the X chromosome that is inherited from the Padre .
Hunter syndrome can be diagnosed at the age of 2 - 4 year . This disease has an relative incidence pace of 1 in 100,000 to 1 in 170,000 male live births . It has been found that around 2,000 the great unwashed worldwide have been afflicted with this disease .
Signs And Symptoms Of Hunter Syndrome
hunting watch syndrome is a multisystem upset so the sign and symptoms are touch on to all the organ and organisation in the body and they are as comply :
The hunter syndrome diagnosis is made on the base of these symptom as well as research lab test and radiological finding . The intervention is also a tricky part because of the amour of all the organisation ; different doctors with specialisation in each organization have to come together for the intervention .
Conclusion
As described above , Orion syndrome is an autosomal recessive or X tie recessionary upset . So , mainly only the male kid is affected but in a few cases distaff child is also affected but very rarely . So the incidence of this disease in males is 1 in 100,000 to 1 in 170,000 alive male childbirths .
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