| sclerostomy | Surgical formation of an external opening in the sclera, primarily in the treatment of glaucoma. (12 Dec 1998) |
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| Scheie | Harold G., U.S. Ophthalmologist, *1909. See: Scheie's syndrome. (05 Mar 2000) |
| scheie's syndrome | <syndrome> 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 carbohydrates). Consequently, the mucopolysaccharides accumulate in the cells 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 syndrome. (09 Oct 1997) |
| Scheie syndrome | <syndrome> Mucopolysaccharidosis (lysosomal disease) in which there is a defect in _ L iduronidase. Fibroblasts from Scheie syndrome patients do not cross correct fibroblasts from Hurler syndrome, although the two conditions are clinically distinct. (18 Nov 1997) |
| Hurler-Scheie syndrome | <syndrome> Although clinically distinct diseases, fibroblasts from patients with Hurler syndrome and with Scheie syndrome do not cross complement in culture, suggesting that the enzyme defect is the same. (18 Nov 1997) |
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