Alpha-L-iduronidase deficiency, severe (medical condition)

A rare inherited biochemical disorder characterized by the accumulation of mucopolysaccharides (glycosaminoglycans) in various body tissues due to insufficient amounts of the enzyme (?-L-diuronidase) needed to break it down. There are three main sub-types of the disorder: Hurler – severe form, Scheie – mild form and Hurler/Scheie form which is intermediate. See also Mucopolysaccharidosis type I Hurler syndrome