mucopolysaccharidosis

(redirected from Scheie syndrome)
Also found in: Dictionary, Thesaurus, Medical.

mucopolysaccharidosis

[¦myü·kō‚päl·ē‚sak·ə·rə′dō·səs]
(medicine)
Any of several inborn metabolic disorders involving mucopolysaccharides; the six types are MPS I, Hurler's syndrome; MPS II, Hunter's syndrome; MPS III, Sanfillipo's syndrome; MPS IV, Morquio's syndrome; MPS V, Scheil's syndrome; and MPS VI, Maroteaux-Lamy's syndrome.
References in periodicals archive ?
Deficiency of this enzyme results into a wide range of phenotypes including Hurler syndrome, Hurler-Scheie syndrome (OMIM 607015) and Scheie syndrome (OMIM 607016), severe, intermediate and mild forms, respectively (3).
Samples n Range Mean [+ or -] SD Healthy adults 40 34.2-158.0 77.6 [+ or -] 30.7 Healthy newborns 25 52.8-135.8 91.0 [+ or -] 27.2 MPS I carriers 4 33.5-76.8 ND (a) Hurler syndrome 4 0-5.2 ND Scheie syndrome 5 0-3.6 ND (a) ND, not determined.
MPS I (also known as Hurler, Hurler-Scheie, and Scheie syndromes) is a life-threatening genetic disease caused by a deficiency of the enzyme alpha-L-iduronidase.