BioScience Dictionary

 
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Found Hunter s Syndrome 3 times.

Displaying results 1 to 10.

1. Hunter's syndrome (mucopolysaccharidosis type II, MPS disorder II, MPS II)
An X-linked disease caused by a deficiency of the enzyme iduronate sulphate sulfatase, which breaks down dermatan sulphate and heparen sulphate, two types of mucopolysaccharides (complex carbohydrate s). Consequently, the mucopolysaccharides accumulate in the cells of the body and cause damage. Patients have the two types of sulfates in their urine and suffer from skeletal defects, retinitis pigmentosa (progressive blindness), skin lesion s, progressive deafness, and heart problems. There are mild and severe forms of the syndrome and presently no cure. Symptoms are treated as they appear. Hunter s syndrome is the most common of a number of related rare genetic mucopolysaccharide disorders.

2. Hurler's syndrome (mucopolysaccharidosis type 1 H, MPS disorder I H, MPS IH, gargoylism, Hurler disease)
A hereditary metabolic disorder caused by a recessive gene which results in a deficiency of the enzyme alpha-L-iduronidase, which breaks down dermatan sulphate and heparan sulphate, two types of mucopolysaccharides (complex carbohydrate s). Consequently, the mucopolysaccharides accumulate in the cell s of the body and cause damage. Symptoms include skeletal deformities, mental retardation, severe coronary artery disease, deafness, and stiff joints, among other things. There are mild and severe forms of the syndrome; the mild form is called Scheie Syndrome , the severe form is called Hurler Syndrome, and children with an intermediate form have Hurler-Scheie Syndrome. Patients with the severe form usually die from respiratory and cardiac failure before the age of ten. There is presently no cure. Hurler s Syndrome is one of a number of related rare genetic mucopolysaccharide disorders, the most common being Hunter s Syndrome .

3. Scheie s Syndrome (mucopolysaccharidosis type 1 S, MPS-IS, MPS disorder I S)
A hereditary metabolic disorder caused by a recessive gene which results in a deficiency of the enzyme alpha-L-iduronidase, which breaks down dermatan sulphate and heparan sulphate, two types of mucopolysaccharides (complex carbohydrate s). Consequently, the mucopolysaccharides accumulate in the cell s of the body and cause damage. Symptoms include skeletal deformities, mental retardation, coronary artery disease, deafness, and stiff joints, among other things. There are mild and severe forms of the syndrome; the severe form is called Hurler Syndrome , the mild form is called Scheie Syndrome, and children with an intermediate form have Hurler-Scheie Syndrome. Patients with the mild form generally suffer little to no mental retardation and survive to adulthood. There is presently no cure. Scheie s Syndrome is one of a number of related rare genetic mucopolysaccharide disorders, the most common being Hunter s Syndrome .