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Scheie Syndrome (MPS IS) exhibits the same defect as Hurler Syndrome, an absence of the enzyme a-L-iduronidase, but is usually milder. It is the mildest form of the mucopolysaccharide diseases. The clinical features of Scheie Syndrome are joint limitations, inguinal hernias, a broad mouth with full lips, increased body hair and clouding of the cornea that occurs early. Affected children also develop juvenile onset of stiff joints, with development of claw hands and deformed feet. As in Morquio syndrome (MPS IV), affected people may develop problems with the aortic valve. Mental ability in those with Scheie Syndrome is maintained and the disorder is compatible with an almost normal lifespan. However, some disabilities such as limitation of joints, blindness or deafness are likely in later decades. |