
Mucopolysaccharidosis Type I
Hurler SyndromeMPS I
Educational overview only. This page summarizes publicly available health information for general understanding and awareness. It does not provide medical advice, diagnosis, or treatment. For personalized guidance or care decisions, consult a qualified healthcare professional.
๐Overview
Mucopolysaccharidosis Type I (MPS I) is a rare inherited lysosomal storage disorder caused by deficiency of the enzyme alpha-L-iduronidase. This leads to accumulation of glycosaminoglycans (GAGs) in various tissues, causing progressive multi-systemic symptoms. MPS I encompasses a spectrum of severity, historically classified into Hurler syndrome (severe), Hurler-Scheie syndrome (intermediate), and Scheie syndrome (mild), but now recognized as a continuous range rather than distinct subtypes.
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Medical codes (for reference)
UMLS CUI: C0023786ICD-10-CM
E76.0
MeSH
D008059
SNOMED CT (US)
75610003
Codes are provided for reference and interoperability. They are not a diagnosis.
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