Hurler Syndrome

**Semantic type:** Disease or Syndrome

**Definition:** An autosomal recessive inherited disorder of mucopolysaccharide metabolism. It is the most severe form of mucopolysaccharidosis type I. It is characterized by deficiency of the enzyme alpha-L-iduronidase resulting in the accumulation of mucopolysaccharides in the tissues.

**Synonyms:** - MPS I H - Mucopolysaccharidosis Type IH