Gourt > Health > Conditions and Diseases > Nutrition and Metabolism Disorders > Inherited > Mucopolysaccharidoses > Hurler Syndrome
Hurler syndrome, also known as mucopolysaccharidosis type I (MPS I) or "Hurler's disease", is a genetic disorder that results in the deficiency of alpha-L iduronidase, which is an enzyme that breaks down mucopolysaccharides. Without this enzyme, the buildup of heparan sulfate and dermatan sulfate occurs in the body (the heart, liver, brain etc.). Symptoms appear during childhood and early death can occur due to organ damage.
MPS I is divided into three subtypes based on severity of symptoms. All three types result from an absence of, or insufficient levels of, the enzyme alpha-L-iduronidase. MPS I H or Hurler syndrome is the most severe of the MPS I subtypes. The other two types are MPS I S or Scheie syndrome and MPS I H-S or Hurler-Scheie syndrome
Hurler's Syndrome is often classified as a lysosomal storage disease and is mechanistically related to Hunter's Syndrome (X-linked recessive).
More on [ Hurler syndrome ]
It's me, Micol - An imaginative personal account written by a parent telling the story of a child's experiences growing up with MPS. Originally written in Italian, it has an English equivalent.
Meta Description: [ vita felice di una bambina di 6 anni, micol addamo, con mucopolissacaridosi (mps). ]
Jenna Marie's Page - The story of Jenna Marie who was born with MPS in 1997. Features updates on her condition and highlights of her life.
MedlinePlus Medical Encyclopedia: Hurler syndrome - Information about Mucopolysaccharidosis type I (MPS I), also known as Alpha-L-iduronate deficiency. Outlines the basic facts such as symptoms, treatments, and prognosis.
Obviously Grace - A mother writes about her experience as a mother of a child who had a form of MPS and died at age eight.
Tyler's Room - Hurler Syndrome - Information on the disease and Tyler's story. Includes an autopsy report.
