ICD10BillableValid for clinical use
ICD-10 Code E76.1: Mucopolysaccharidosis, type II
Reviewed by HealthAssure Clinical TeamUpdated 26 May 2026
What is this code?
ICD-10 codes are diagnosis classification codes used in healthcare records, reporting, coding workflows, and billing support. 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 ii in a patient's medical record.
- May appear in hospital records, claims, referrals, and clinical documentation.
- This code can be used as a clinically usable diagnosis entry in standardized coding workflows.
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.
Code hierarchy
chapter
4Endocrine, nutritional and metabolic diseases (E00-E89)
block
E70-E88Metabolic disorders
category
E76Disorders of glycosaminoglycan metabolism
currentE76
Official coding notes
Inclusion terms
- Hunter's syndrome
Where you may see this code
You may see this code in hospital records, discharge summaries, insurance claims, encounter documentation, referrals, or other healthcare billing and coding records.
Related specialists
EndocrinologistGeneral Physician
Related codes
Sibling codes
Coverage-related procedures and services
CPT 75716high
ANGIOGRAPHY, EXTREMITY, BILATERAL, RADIOLOGICAL SUPERVISION AND INTERPRETATION
Source: CMS coverage guidance
CPT 75710high
ANGIOGRAPHY, EXTREMITY, UNILATERAL, RADIOLOGICAL SUPERVISION AND INTERPRETATION
Source: CMS coverage guidance
CPT 75705high
ANGIOGRAPHY, SPINAL, SELECTIVE, RADIOLOGICAL SUPERVISION AND INTERPRETATION
Source: CMS coverage guidance
CPT 75630high
AORTOGRAPHY, ABDOMINAL PLUS BILATERAL ILIOFEMORAL LOWER EXTREMITY, CATHETER, BY SERIALOGRAPHY, RADIOLOGICAL SUPERVISION AND INTERPRETATION
Source: CMS coverage guidance
CPT 75625high
AORTOGRAPHY, ABDOMINAL, BY SERIALOGRAPHY, RADIOLOGICAL SUPERVISION AND INTERPRETATION
Source: CMS coverage guidance
CPT 93598high
CARDIAC OUTPUT MEASUREMENT(S), THERMODILUTION OR OTHER INDICATOR DILUTION METHOD, PERFORMED DURING CARDIAC CATHETERIZATION FOR THE EVALUATION OF CONGENITAL HEART DEFECTS (LIST SEPARATELY IN ADDITION TO CODE FOR PRIMARY PROCEDURE)
Source: CMS coverage guidance
Mapped diagnoses and classifications
SNOMED_CT 192795000high
Cerebral degeneration in Hunter's disease
Source: SNOMED International ICD-10 map
SNOMED_CT 5667009high
Hunter's syndrome, mild form
Source: SNOMED International ICD-10 map
SNOMED_CT 73146005high
Hunter's syndrome, severe form
Source: SNOMED International ICD-10 map
SNOMED_CT 70737009high
Mucopolysaccharidosis, MPS-II
Source: SNOMED International ICD-10 map
Coding guidelines
Compatibility
Legacy and official ICD code match exactly.
Legacy codes
E76.1
Common synonyms
Mucopolysaccharidosis, type II
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.