E76.01 ICD-10-CM Code: Hurler's syndrome
E76.01 maps to CMS-HCC V28 49. A source-labeled RAF reference is available. Confirm the documented diagnosis and applicable coding requirements. MEAT criteria · RAF Calculator · HCC Buddy coding tools
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FY 2026 Apr update / Endocrine, nutritional and metabolic diseases (E00-E89) / Metabolic disorders (E70-E88)
E76.01
Billable / SpecificICD-10-CMOfficial ICD-10-CMCodebook guidanceHurler's syndrome
A severe inherited metabolic disorder (mucopolysaccharidosis type I) where the body cannot break down certain complex sugars, causing progressive damage to multiple organs including the heart, bones, and brain.
Buddy Insight
Hurler syndrome (mucopolysaccharidosis type I-H) is a severe, progressive lysosomal storage disorder caused by deficiency of alpha-L-iduronidase, leading to accumulation of dermatan sulfate and heparan sulfate.
CMS-HCC V28
MappedHCC 49
Coefficient HCC 49: 9.256 (Community Non-Dual Aged (CNA))
CMS-HCC V24
HistoricalHistorical
Not used for CY2026 payment
ACA/HHS
MappedHCC 026
Code-level coefficient reference
ESRD/PACE
MappedHCC 23
Code-level coefficient reference
RXHCC
MappedHCC 41
Code-level coefficient reference
Code Book Path
Inclusion Terms
OfficialNo inclusion terms are included in this display for E76.01. Check the code and parent instructions in the Code Book.
Excludes 2
Official- Ehlers-Danlos syndromes (Q79.6-)Inherited from E70-E88
Related Codes
Includes
OfficialNo Includes notes are included in this display for E76.01. Check the code and parent instructions in the Code Book.
Excludes 1
Official- transitory endocrine and metabolic disorders specific to newborn (P70-P74)Inherited from E00-E89, E70-E88
- androgen insensitivity syndrome (E34.5-)Inherited from E00-E89, E70-E88
- congenital adrenal hyperplasia (E25.0)Inherited from E00-E89, E70-E88
- hemolytic anemias attributable to enzyme disorders (D55.-)Inherited from E00-E89, E70-E88
- Marfan syndrome (Q87.4-)Inherited from E00-E89, E70-E88
- 5-alpha-reductase deficiency (E29.1)Inherited from E00-E89, E70-E88
Code First
OfficialNo Code First sequencing instructions are included in this display for E76.01. Check the code and parent instructions in the Code Book.
Use Additional
OfficialNo Use Additional Code instructions are included in this display for E76.01. Check the code and parent instructions in the Code Book.
Code Also
OfficialNo Code Also instructions are included in this display for E76.01. Check the code and parent instructions in the Code Book.
Buddy Documentation Tip
MEAT Support
Audit Caution
Common Mistakes
Current with CMS: FY2026 ICD-10-CM Apr 1 update (effective Apr 1 – Sep 30, 2026) · CMS-HCC V28, 100% phased in for payment year 2026. FY2027 code set already staged for October 1, 2026. How HCC Buddy stays current →
Is E76.01 an HCC code?
Yes. E76.01 (Hurler's syndrome) maps to HCC 49, Specified Lysosomal Storage Disorders under the CMS-HCC V28 risk adjustment model, with a source-labeled community non-dual aged reference coefficient of 9.256. Source-labeled code-level coefficients are references, not member totals. Actual contribution depends on complete member context, hierarchy and cleanup rules, interactions, and model year. HCC Buddy's RAF Calculator supports CMS-HCC V28 PY2026 and shows no score unless every required source and calculation check passes. It is billable for payment year 2026.
Coder answer: E76.01 is billable and maps to V28 HCC 49, Specified Lysosomal Storage Disorders. Open it in the Code Book for the tabular path, RAF, and MEAT checklist.
- Code
- E76.01
- Description
- Hurler's syndrome
- HCC (V28)
- HCC 49 — Specified Lysosomal Storage Disorders
- RAF reference coefficient
- 9.256
- Billable
- Yes
- Payment year
- 2026
HCC Category Mapping
These are source-labeled model coefficients, not member totals. A category may still be removed by hierarchy or model cleanup rules. Weights are not directly comparable across models: CMS-HCC V28 and V24 use Community, Non-Dual, Aged; ESRD uses the dialysis continuing-enrollee model; RxHCC is the Part D continuing-enrollee, non-low-income, aged weight (a larger scale than CMS-HCC). ACA/HHS has no single weight — it varies by metal level. Actual per-patient RAF contribution depends on member context, hierarchy and cleanup rules, interactions, and the model year used by the payer. V28 is the CMS-HCC model phased in over payment years 2024–2026; V24 remains available for historical review.
Work E76.01 in the Code Book — tabular path, V28 RAF reference, and MEAT checklist →
MEAT review for E76.01
For E76.01, confirm that the documentation supports the diagnosis and meets the applicable coding, encounter, program and payer requirements. MEAT (Monitor, Evaluate, Assess, or Treat) is a review mnemonic, not a universal CMS coding rule.
- MMonitor: signs, symptoms, disease progression, or lab trending documented in the note
- EEvaluate: test results, medication response, or physical findings reviewed by the provider
- AAssess: explicit mention in the assessment or plan with acknowledgment of status
- TTreat: medication, referral, procedure, therapy, or counseling tied to the diagnosis
Coder workflow notes
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What This Code Means
E76.01 is the ICD-10-CM diagnosis code for hurler's syndrome. A severe inherited metabolic disorder (mucopolysaccharidosis type I) where the body cannot break down certain complex sugars, causing progressive damage to multiple organs including the heart, bones, and brain. E76.01 sits in the ICD-10-CM chapter for endocrine, nutritional and metabolic diseases (e00-e89), within the section covering metabolic disorders (e70-e88).
Under the CMS-HCC V28 risk adjustment model, E76.01 maps to Specified Lysosomal Storage Disorders (HCC 49) with a source-labeled community, non-dual, aged reference coefficient of 9.256. For CY2026 non-PACE Medicare Advantage, CMS uses 100% of the 2024 CMS-HCC model (V28). PACE uses a separate model blend. Source-labeled code-level coefficients are references, not member totals. Actual contribution depends on complete member context, hierarchy and cleanup rules, interactions, and model year. HCC Buddy's RAF Calculator supports CMS-HCC V28 PY2026 and shows no score unless every required source and calculation check passes.
Document the age of onset and severity to support medical necessity for specialized treatments. For E76.01, confirm that the documentation supports the diagnosis and meets the applicable coding, encounter, program and payer requirements. MEAT (Monitor, Evaluate, Assess, or Treat) is a review mnemonic, not a universal CMS coding rule. When documentation is ambiguous, coders should issue a provider query rather than assume the highest-specificity variant.
HCC Buddy maintains structured V28 and V24 mapping, source-labeled coefficient references, and MEAT documentation criteria for E76.01 sourced directly from the CMS-HCC risk adjustment model files and the CMS ICD-10-CM code set.
Coding Tips
- •Document the age of onset and severity to support medical necessity for specialized treatments
- •Code associated complications such as cardiac valve disease, skeletal abnormalities, or developmental delay separately
Clinical Significance
Hurler syndrome (mucopolysaccharidosis type I-H) is a severe, progressive lysosomal storage disorder caused by deficiency of alpha-L-iduronidase, leading to accumulation of dermatan sulfate and heparan sulfate. It is the most severe form of MPS I, with life-threatening cardiac, skeletal, and neurological complications typically requiring hematopoietic stem cell transplant or enzyme replacement therapy.
Documentation Requirements
- ✓Specific diagnosis of Hurler syndrome (MPS I-H) as distinct from Hurler-Scheie or Scheie variants
- ✓Confirmatory enzyme assay showing alpha-L-iduronidase deficiency or genetic testing with IDUA gene mutation
- ✓Current disease manifestations: cardiac (valvular disease), skeletal (dysostosis multiplex), neurological (cognitive decline), corneal clouding
- ✓Treatment status: enzyme replacement therapy (laronidase), hematopoietic stem cell transplant history, or supportive care
- ✓Functional status assessment and disease progression documentation
Commonly Confused Codes
- •E76.02: Hurler-Scheie syndrome: intermediate MPS I phenotype, less severe than Hurler
- •E76.03: Scheie's syndrome: mildest MPS I form with normal intelligence and longer survival
- •E76.1: Mucopolysaccharidosis type II (Hunter syndrome): different enzyme deficiency (iduronate-2-sulfatase), X-linked
- •E76.3: Mucopolysaccharidosis, unspecified: use only when the specific MPS type is not documented
- •E76.29: Other mucopolysaccharidoses: for MPS types not individually coded (e.g., MPS VI Maroteaux-Lamy, MPS VII Sly)