E76.02
BillableHurler-Scheie syndrome
Last updated: FY2026 ICD-10-CM (Oct 1, 2025 – Sep 30, 2026) | CMS-HCC V28 (100% phase-in, PY2026)
Is E76.02 an HCC code?
Yes. E76.02 maps to Lysosomal Storage Disorders under the CMS-HCC V28 risk adjustment model (and Other Significant Endocrine and Metabolic Disorders under V24).
HCC Category Mapping
RAF weights shown are the community, non-dual, aged base weights from the CMS risk adjustment model file. Actual per-patient RAF contribution depends on member segment, interactions, and the model year used by the payer. V28 is the CMS-HCC model phased in over payment years 2024–2026; V24 remains in use during the transition and for historical data.
MEAT Criteria for E76.02
For E76.02 to count as a valid HCC diagnosis in a given encounter, the provider's documentation must show MEAT: Monitor, Evaluate, Assess, or Treat. A diagnosis from a prior year does not carry forward automatically — it has to be re-documented and supported each calendar year.
- 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
Only one of M/E/A/T is required to support the code, but the documentation must be specific enough to show that the provider actually addressed E76.02 during that encounter — not just copy-forwarded from a problem list.
What This Code Means
E76.02 is the ICD-10-CM diagnosis code for hurler-scheie syndrome. An intermediate form of mucopolysaccharidosis type I with slower progression than Hurler's syndrome, causing progressive organ and skeletal damage with variable neurological involvement. E76.02 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, E76.02 maps to Lysosomal Storage Disorders (HCC 49) with a community, non-dual, aged base RAF weight of 0.226. Under the older CMS-HCC V24 model, E76.02 maps to Other Significant Endocrine and Metabolic Disorders (HCC 23) with a community, non-dual, aged base RAF weight of 0.230. V28 is the CMS-HCC risk adjustment model that reached 100% phase-in for payment year 2026, replacing V24 which was used during the PY2024–PY2025 transition.
Distinguish from Hurler's syndrome (E76.01) by documenting the intermediate clinical course and age of onset. Because E76.02 maps to a payment HCC, the provider's documentation must satisfy MEAT criteria (Monitor, Evaluate, Assess, or Treat) for the encounter to count toward the patient's Medicare Advantage risk adjustment score. 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, RAF weights, and MEAT documentation criteria for E76.02 sourced directly from the CMS-HCC risk adjustment model files and the CMS ICD-10-CM code set.
Coding Tips
- •Distinguish from Hurler's syndrome (E76.01) by documenting the intermediate clinical course and age of onset
- •Link to specific organ involvement such as cardiac, skeletal, or respiratory complications
Clinical Significance
Hurler-Scheie syndrome (MPS I-H/S) is an intermediate phenotype of mucopolysaccharidosis type I, with onset typically between ages 3-8 years and survival into the teens or twenties. It causes significant morbidity through joint stiffness, cardiac valve disease, and hepatosplenomegaly but generally spares cognitive function, distinguishing it from Hurler syndrome.
Documentation Requirements
- ✓Specific documentation of Hurler-Scheie (MPS I-H/S) intermediate phenotype
- ✓Confirmatory enzyme assay or IDUA gene mutation analysis
- ✓Current organ system involvement: cardiac, musculoskeletal, hepatic, respiratory, ophthalmologic
- ✓Treatment plan including enzyme replacement therapy (laronidase/Aldurazyme) or surgical interventions
- ✓Cognitive function status to support intermediate (not severe) phenotype classification