What Is Hurler Syndrome?
Hurler Syndrome is a importantly rarefied familial aesculapian consideration in which the body is unable to wear out down loot molecules call glycosaminoglycans result in a gradual buildup of this molecule resulting in a variety of symptom and complications . In this Hurler Syndrome disease , the body has a malfunctioning enzyme called lysosomal alpha - L - iduronidase which is not able to erupt down the sugar molecule make Hurler Syndrome .
What Are The Causes Of Hurler Syndrome?
As posit , Hurler Syndrome is stimulate due to a malfunctioning enzyme called lysosomal alpha - L - iduronidase which facilitates equipment failure of the sugar speck called glycosaminoglycans . These particle are present throughout the body and are normally located around the joints or in the mucous . With the enzyme not being able to breakdown the sugar molecule , there is a gradual buildup of glycosaminoglycans in the body which bug out to make significant equipment casualty to the organ of the body to include the heart and may cause significant complications . As tell , Hurler Syndrome is a genic medical condition which is autosomal recessionary meaning that both parents should post the defective gene in Holy Order for the progeny to grow the disease .
What Are The Symptoms Of Hurler Syndrome?
Symptoms Of Hurler Syndrome Include:
How Is Hurler Syndrome Diagnosed?
Some Of The Measures Utilized To Diagnose Hurler Syndrome Are:
These above tests can definitively sustain the diagnosis of Hurler Syndrome .
What Are The Treatments For Hurler Syndrome?
There is no clear make out treatment for Hurler Syndrome and treatments are usually take aim at assure the symptoms ; however , there are some investigational treatments which have usher some effectiveness in discussion of Hurler Syndrome and these measure are :
Enzyme Replacement Therapy : In this mode of treatment , the offending enzyme is supercede with a healthy enzyme . This is normally done through a medication called laronidase which is administered intravenously .
Bone Marrow Transplant : This method of handling has also shown great hope in avail with the symptoms of Hurler Syndrome .
aside from these treatments , other treatments are dependent on the organs affected by Hurler Syndrome .
Hurler Syndrome Prognosis And Life Expectancy
Overall medical prognosis of Hurler Syndrome is extremely piteous and affected youngster may not be capable to hold out for long .
