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  • Scheie syndrome
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  • Scheie operation-thermal sclerostomy
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  • hurler-scheie syndrome
    Çæ·¯ »þÀÌ¿¡ÁõÈıº, Hurler-Scheie ÁõÈıº
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  • Hurler-Scheie syndrome
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Scheie Harold G., U.S. Ophthalmologist, *1909.
See: Scheie's syndrome.
(05 Mar 2000)
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)
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)
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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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Scheie's operation 1. scleral cauterization with peripheral iridectomy for treatment of glaucoma. 2. a technique for needling and aspiration of cataract.
Ãâó: www.mercksource.com/pp/us/cns/cns_hl_dorlands.jspz...
Scheie's o. 1. scleral cauterization with peripheral iridectomy for treatment of glaucoma.  2. a technique for needling and aspiration of cataract.
Ãâó: www.mercksource.com/pp/us/cns/cns_health_library.j...
Scheie's s. a relatively mild allelic variant of Hurler's syndrome and the mildest of the three allelic disorders of mucopolysaccharidosis I, characterized by corneal clouding, claw hand, involvement of the aortic valve, somewhat coarse facies with a broad mouth, genu valgum, and pes cavus. Stature, intelligence, and life span are normal; it is caused by a deficiency of L-iduronidase. Called also mucopolysaccharidosis IS; formerly called mucopolysaccharidosis V.
Ãâó: www.mercksource.com/pp/us/cns/cns_health_library.j...
Scheie's syndrome see under syndrome.
Ãâó: www.mercksource.com/pp/us/cns/cns_health_library.j...
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