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Hunter syndrome is also known as mucopolysaccharidosis character II . huntsman syndrome is a disease due to deficiency of an enzyme name iduronate-2 - sulfatase ( I2S ) , which is a lysosomal enzyme . It is an X - linked recessive disease . Due to deficiency of iduronate-2 - sulfatase , there is accumulation of dermatan sulphate and Liquaemin sulfate ( both are type of mucopolysaccharides ) in the eubstance tissue and this causes freakishness in the major organs like heart , central nervous system and respiratory system .
Alternative Treatment For Hunter Syndrome
Hunter syndrome is a non - curable disease . There are few emerge alternate discourse choice for this disease-
Enzyme Replacement Therapy (ERT)
A purified form of the enzyme iduronate-2 - sulfatase that is wanting in this disease is available as idursulfase and this can be used to reduce the severity of Hunter syndrome . dole out this drug into the body of patient can be helpful to breakdown the mucopolysaccharide . But this enzyme can not cut across the pedigree brain barrier that covers the brain and so if the disease is wicked and affects the brainiac , this treatment method will not be very useful.(1 ) ( 2 )
After judicature of ERT the patient ’s urinary GAG excretory product level must be supervise . Also the exercising weight of the patient role must be kept in bridle to adjust the sexually transmitted disease accordingly . Usually ERT is commit in the venereal infection of 0.5 mg / kg per week . Chances are there that ERT might cause reaction . Two type of reactions can come , one that occurs at the time of extract and other occurs after 12 hour of infusion . The immediate reaction presents as chill , feverishness and urticaria . When these signs are seen , the infusion must be finish promptly and antihistamines must be administer . The extract can be resume after 30 minutes . To prevent the chances of chemical reaction , the aesculapian health worker can premedicate the patient with antihistamines or acetaminophen .
When the reaction take place after 12 hour of infusion , the presenting signs are reckless and wheezing . The rash can be cover with corticoid , antihistamines or acetaminophen . If wheeze is present , it can be treat with the aid of O supplementation or bronchodilator or both .
Anti - incendiary drug – Using anti - inflammatory drugs helps to shorten the rigourousness of rubor and help in improving the timbre of life of the patient .
Hematopoietic Stem Cell Transplantation(HSCT)- In HSCT , the donation can be of corduroy blood , pearl marrow or peripheral blood stem cellular phone from the healthy presenter . But it was find that patients who were treat with HSCT had high mortality pace .
Gene Editing Therapy – This is the most late advancement for care for hunting watch syndrome . It for good alters the DNA of the patient role by edit the diseased chromosome with normal one .
Other Types Of Mucopolysaccharidosis
Mucopolysaccharidosis Type I ( MPS I)-
On the groundwork of severeness of the disease MPS I , it can be separate into three subtypes- Hurler syndrome , Scheie syndrome and Hurler - Scheie syndrome . Hurler and Scheie syndromes are the two extremities of the disease and Hurler - Scheie syndrome falls in between the two .
MPS I is make due to deficiency of alpha - liter - iduronidase . The three subtypes are differentiated on the footing of clinical symptom and severity . Hurler syndrome ( MPS 1 - heat content ) is a multisystem disease and it results in former death of the minor . It is name at the age of 4 to 18 months .
Scheie syndrome is the meek amongst all the type of mucoploysaccharidosis . The symptoms of this eccentric are come across after the age of five years . The kid suffering from this disease usually have normal intelligence , normal growth and stature and good life-time anticipation . The symptoms include- carpal tunnel syndrome , sore joints , glaucoma and aortic valve disease .
Hurler - Scheie Syndrome ( MPS I H / S)-
It is an average type of disease . It is neither spartan nor meek . There is little or no abnormalcy have-to doe with to noetic capacitance . The symptom appear between the historic period of three to eight years . The symptom include , corneal clouding , common facial features , scant stature , joint stiffness and hepatosplenomegaly . The patient can survive up to adulthood .
Mucopolysaccharidosis type 3- also called as Sanfilippo syndrome
Mucopolysaccharidosis type 4- also address as Morquio syndrome
Mucopolysaccharidosis type 6- also call as Maroteaux - Lamy syndrome
Mucopolysaccharidosis type 7- also known as Sly syndrome
Mucopolysaccharidosis eccentric 9- also have it off as Hyaluronidase deficiency
Conclusion
The alternative discussion options for hunter syndrome are Enzyme Replacement Therapy ( ERT ) , anti - inflammatory drug , Hematopoietic Stem Cell Transplantation ( HSCT ) and cistron editing therapy .
Enzyme Replacement Therapy ( ERT ) for hunter syndrome admit intervention with idursulfase . Anti - rabble-rousing drugs like adrenal cortical steroid are used to come down the intensiveness of inflammation . HSCT is not a preferable choice because it has been detect that it increases chances of fatality rate . Also , graft versus legion reactions are also noted in the patients . Along with all this , social financial backing and transmitted support is very necessary for betterment of the affected role .
References-
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