ICD10
ICD-10 Code E76.0: Mucopolysaccharidosis, type I
Reviewed by HealthAssure Clinical TeamUpdated 26 May 2026
What is this code?
ICD-10 entries help standardize how diagnoses are organized for coding, reporting, analytics, and documentation. This code sits within the broader ICD-10 area for Endocrine, nutritional and metabolic diseases (E00-E89).
When is it used?
- May be used when a clinician documents mucopolysaccharidosis, type i in a patient's medical record.
- May appear in hospital records, claims, referrals, and clinical documentation.
- This code may act more like a grouping or parent code, so a more specific child code may be used in final documentation when available.
What it does not mean
- A code alone does not explain severity, treatment plan, or outcome.
- A medical code should not be treated as a substitute for a doctor's diagnosis or advice.
- This entry may represent a broader category rather than the most specific billable code.
Code hierarchy
chapter
4Endocrine, nutritional and metabolic diseases (E00-E89)
block
E70-E88Metabolic disorders
category
E76Disorders of glycosaminoglycan metabolism
currentE76
Where you may see this code
You may see this entry in coding references, medical records, or claims workflows when a broader diagnosis category is being reviewed before a more specific code is chosen.
Related specialists
EndocrinologistGeneral Physician
Sibling codes
Mapped diagnoses and classifications
SNOMED_CT 254069004high
Dysostosis multiplex
Source: SNOMED International ICD-10 map
SNOMED_CT 279081001high
Dysostosis multiplex group
Source: SNOMED International ICD-10 map
SNOMED_CT 75610003high
Mucopolysaccharidosis, MPS-I
Source: SNOMED International ICD-10 map
SNOMED_CT 65327002high
Mucopolysaccharidosis, MPS-I-H
Source: SNOMED International ICD-10 map
SNOMED_CT 26745009high
Mucopolysaccharidosis, MPS-I-H/S
Source: SNOMED International ICD-10 map
SNOMED_CT 73123008high
Mucopolysaccharidosis, MPS-I-S
Source: SNOMED International ICD-10 map
Coding guidelines
Compatibility
Legacy code aligns to an official FY 2026 category. Frontend should resolve to the official category page and surface the billable child codes.
Legacy codes
E76.0
Replacement codes
E76.01 — Hurler's syndromeE76.02 — Hurler-Scheie syndromeE76.03 — Scheie's syndrome
Common synonyms
Mucopolysaccharidosis, type I
Frequently asked questions
About this content
This page is prepared by HealthAssure's clinical team using official coding standards from ICD-10. AI tools assist with drafting explanations, which are then reviewed and verified by healthcare professionals for accuracy. This content is for informational purposes and does not replace professional medical advice. Meet our team.