📋Overview

This condition occurs when the body lacks a specific enzyme called iduronate-2-sulfatase, which is necessary to break down complex sugar molecules. Without this enzyme, these substances accumulate in various tissues and organs throughout the body, leading to progressive cellular damage.

The clinical presentation of this disorder exists on a spectrum of severity and can impact multiple systems as the accumulation of molecules continues over time. Because the gene associated with the condition is located on the X chromosome, it is observed almost exclusively in male patients.

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.

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Medical codes (for reference)

UMLS CUI: C0026705
ICD-10-CM
E76.1
MeSH
D016532
SNOMED CT (US)
70737009

Codes are provided for reference and interoperability. They are not a diagnosis.

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