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E76.211

Billable

Morquio B mucopolysaccharidoses

Last updated: FY2026 ICD-10-CM (Oct 1, 2025 – Sep 30, 2026) | CMS-HCC V28 (100% phase-in, PY2026)

Is E76.211 an HCC code?

Yes. E76.211 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

V28HCC 49Lysosomal Storage Disorders
0.226
V24HCC 23Other Significant Endocrine and Metabolic Disorders
0.230
ESRDHCC 23Other Significant Endocrine and Metabolic Disorders
0.000
RxHCCHCC 41Lysosomal Storage Disorders
0.000

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 E76.211

For E76.211 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 E76.211 during that encounter — not just copy-forwarded from a problem list.

What This Code Means

E76.211 is the ICD-10-CM diagnosis code for morquio b mucopolysaccharidoses. Morquio B mucopolysaccharidosis is a rare inherited metabolic disorder similar to Morquio A but caused by a different enzyme deficiency, resulting in skeletal abnormalities and progressive physical limitations. E76.211 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, E76.211 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, E76.211 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.

Morquio B is less common than Morquio A; verify the specific enzyme deficiency is documented to ensure correct code selection. Because E76.211 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 E76.211 sourced directly from the CMS-HCC risk adjustment model files and the CMS ICD-10-CM code set.

Coding Tips

  • Morquio B is less common than Morquio A; verify the specific enzyme deficiency is documented to ensure correct code selection
  • Document the degree of skeletal involvement and any systemic complications for complete clinical picture

Clinical Significance

Morquio B mucopolysaccharidosis (MPS IVB) is caused by beta-galactosidase deficiency, leading to keratan sulfate accumulation with a clinical phenotype similar to Morquio A but generally milder. Distinguishing Morquio B is important because it shares an enzyme deficiency with GM1 gangliosidosis, and treatment approaches may differ from Morquio A.

Documentation Requirements

  • Specific diagnosis of Morquio B syndrome (MPS IVB) documented
  • Confirmatory enzyme assay showing beta-galactosidase deficiency or GLB1 gene mutation analysis
  • Documentation distinguishing this from GM1 gangliosidosis (which shares the same enzyme deficiency but has different clinical features)
  • Skeletal manifestations and severity assessment
  • Current treatment and management plan

Commonly Confused Codes

Code Hierarchy

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