Hurler syndrome classification

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Classification

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).