Medical Definition of Type IS mucopolysaccharidosis

1. 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)

Lexicographical Neighbors of Type IS Mucopolysaccharidosis

type III hyperlipoproteinaemia
type III hypersensitivity reaction
type III mucopolysaccharidosis
type II acrocephalosyndactyly
type II cells
type II collagen
type II diabetes
type II dip
type II error
type II errors
type II familial hyperlipoproteinaemia
type II hyperlipoproteinaemia
type II interferon
type II mortality
type II mucopolysaccharidosis
type IS mucopolysaccharidosis (current term)
type IV allergic reaction
type IV familial hyperlipoproteinaemia
type IV prepilin peptidase
type I H/S mucopolysaccharidosis
type I acrocephalosyndactyly
type I allergic reaction
type I cells
type I collagen
type I diabetes
type I diabetes mellitus
type I dip
type I error

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