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Abstract

CASE REPORT ON HUNTER SYNDROME: A REVIEW

Vaishnavi L. R.*, Anju Deepak Unnithan and Supriya S.

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Abstract

Hunter syndrome is an X linked recessive mucopolysaccharidosis (type II) caused by the deficiency of iduronate 2-sulfatase. This in turn leads to the accumulation of glycosaminoglycans, dermatan and heparan sulfate. The intracellular and extracellular accumulation of these substances lead to multisystemic organ abnormality. It is a rare syndrome with a very low prevalence of 1.3:100 000 male live births. Hunter syndrome is one of a group of diseases called mucopolysaccharidoses.

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