Scheie's syndrome

См. также в других словарях:

• Scheie's syndrome — ▪ pathology also called  Mucopolysaccharidosis I S , or  Mps I S        uncommon hereditary metabolic disease characterized by clawing of the hands, corneal clouding, incompetence of the aortic valve of the heart, and painful nerve compression in …   Universalium

• Scheie operation syndrome — (sha) [Harold Glendon Scheie, American ophthalmologist, 1909–1990] see under operation and syndrome …   Medical dictionary

• Scheie syndrome — shī n an autosomal recessive mucopolysaccharidosis similar to Hurler s syndrome but less severe that is characterized by clouding of the cornea, slight deformity of the extremities, and disease of the aorta but not by mental retardation or early… …   Medical dictionary

• Scheie syndrome — (also known as MPS V) ) is less severe version of Hurler s disease. It is a condition characterized by corneal clouding, facial dysmorphism, and normal lifespan [1] but unlike Hurler s, disease has normal intellect and .[2] See also Sanfilippo… …   Wikipedia

• Syndrome de Hurler-Scheie-Hurler/Scheie — Syndrome de Hurler Syndrome de Hurler Autre nom Mucopolysaccharidose de type 1 Référence MIM …   Wikipédia en Français

• Syndrome, Hurler — An inherited error of metabolism in which there is deficiency of the enzyme alpha L iduronidase which normally breaks down molecules called mucopolysaccharides. Without the activity of this enzyme, there is an abnormal accumulation of… …   Medical dictionary

• syndrome — The aggregate of symptoms and signs associated with any morbid process, and constituting together the picture of the disease. SEE ALSO: disease. [G. s., a running together, tumultuous concourse; (in med.) a concurrence of symptoms, fr. syn,… …   Medical dictionary

• Scheie syndrome — Mucopolysaccharidosis (lysosomal disease) in which there is a defect in a L iduronidase. Fibroblasts from Scheie syndrome patients do not cross correct fibroblasts from Hurler&’s disease, although the two conditions are clinically distinct …   Dictionary of molecular biology

• Scheie — Harold G., U.S. ophthalmologist, *1909. See S. syndrome …   Medical dictionary

• síndrome de Scheie — Eng. Scheie s syndrome Mucopolisacaridosis que se transmite de forma autosómica recesiva caracterizada por inteligencia normal, cara ensanchada, manos cortas, uñas deformadas, sordera relativa y posible cardiopatía. A nivel ocular presentan… …   Diccionario de oftalmología

• Hurler syndrome — An inherited error of metabolism in which there is deficiency of the enzyme alpha L iduronidase which normally breaks down molecules called mucopolysaccharides. Without the activity of this enzyme, there is an abnormal accumulation of… …   Medical dictionary