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E76.210 ICD-10-CM Code: Morquio A mucopolysaccharidoses

E76.210 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 · free HCC coding tools

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Code lookupE76.210

FY 2026 Apr update / Endocrine, nutritional and metabolic diseases (E00-E89) / Metabolic disorders (E70-E88)

E76.210

Billable / SpecificICD-10-CMOfficial ICD-10-CMCodebook guidance

Morquio A mucopolysaccharidoses

Morquio A mucopolysaccharidosis is a rare inherited metabolic disorder characterized by severe skeletal deformities, short stature, and progressive joint and spine problems due to accumulation of complex sugars.

Buddy the Bee presenting code insight

Buddy Insight

Morquio A mucopolysaccharidosis (MPS IVA) is caused by deficiency of N-acetylgalactosamine-6-sulfatase (GALNS), leading to accumulation of keratan sulfate and chondroitin-6-sulfate.

OverviewRisk ModelsCode BookDocumentationAuditNotesEdition comparison

CMS-HCC V28

HCC 49

Coefficient HCC 49: 9.256 (Community Non-Dual Aged (CNA))

CMS-HCC V24

Historical

Historical

Not used for CY2026 payment

ACA/HHS

HCC 026

Code-level coefficient reference

ESRD/PACE

HCC 23

Code-level coefficient reference

RXHCC

HCC 41

Code-level coefficient reference

Code Book Path

Official
E76.2Other mucopolysaccharidosesE76.21Morquio mucopolysaccharidosesE76.210Morquio A mucopolysaccharidoses

Inclusion Terms

Official
  • Classic Morquio syndrome
  • Morquio syndrome A
  • Mucopolysaccharidosis, type IVA

Excludes 2

Official
  • Ehlers-Danlos syndromes (Q79.6-)Inherited from E70-E88

Related Codes

Official
E76.211Morquio B mucopolysaccharidosesE76.219Morquio mucopolysaccharidoses, unspecified

Includes

Official

No Includes notes are included in this display for E76.210. 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

Official

No Code First sequencing instructions are included in this display for E76.210. Check the code and parent instructions in the Code Book.

Use Additional

Official

No Use Additional Code instructions are included in this display for E76.210. Check the code and parent instructions in the Code Book.

Code Also

Official

No Code Also instructions are included in this display for E76.210. Check the code and parent instructions in the Code Book.

Buddy Documentation Tip

HCC Buddy guidance
Specific diagnosis of Morquio A syndrome (MPS IVA) documented
Confirmatory enzyme assay showing GALNS deficiency or genetic testing with GALNS gene mutation
Skeletal manifestations: short trunk dwarfism, pectus carinatum, genu valgum, odontoid hypoplasia
Cervical spine stability assessment (risk of atlantoaxial subluxation/spinal cord compression)

MEAT Support

HCC Buddy guidance
Specific diagnosis of Morquio A syndrome (MPS IVA) documented
Confirmatory enzyme assay showing GALNS deficiency or genetic testing with GALNS gene mutation
Skeletal manifestations: short trunk dwarfism, pectus carinatum, genu valgum, odontoid hypoplasia
Cervical spine stability assessment (risk of atlantoaxial subluxation/spinal cord compression)

Audit Caution

HCC Buddy guidance
Confusing Morquio A (GALNS deficiency) with Morquio B (beta-galactosidase deficiency) — different enzymes but similar clinical presentation
Using the unspecified Morquio code E76.219 when enzyme or genetic testing clearly identifies subtype A
Coding only the skeletal manifestations without the underlying MPS IVA diagnosis
Failing to document the critical distinction of preserved intelligence in Morquio vs. other MPS types

Common Mistakes

HCC Buddy guidance
E76.211 — Morquio B (MPS IVB): different enzyme deficiency (beta-galactosidase), same skeletal phenotype
E76.219 — Morquio, unspecified: use only when A vs. B subtype is not documented
E76.1 — Hunter syndrome (MPS II): has skeletal features but also hepatosplenomegaly and potential neurodegeneration
Q77.4 — Achondroplasia: skeletal dysplasia that may mimic Morquio radiographically but is a different entity

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 E76.210 an HCC code?

Yes. E76.210 (Morquio A mucopolysaccharidoses) 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: E76.210 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
E76.210
Description
Morquio A mucopolysaccharidoses
HCC (V28)
HCC 49 — Specified Lysosomal Storage Disorders
RAF reference coefficient
9.256
Billable
Yes
Payment year
2026

HCC Category Mapping

V28HCC 49, Specified Lysosomal Storage Disorders
9.256
ESRDHCC 23, Other Significant Endocrine and Metabolic Disorders
Not separately weighted
RxHCCHCC 41, Lysosomal Storage Disorders
Not separately weighted

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 E76.210 in the Code Book — tabular path, V28 RAF reference, and MEAT checklist →

MEAT review for E76.210

For E76.210, 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

E76.210 is the ICD-10-CM diagnosis code for morquio a mucopolysaccharidoses. Morquio A mucopolysaccharidosis is a rare inherited metabolic disorder characterized by severe skeletal deformities, short stature, and progressive joint and spine problems due to accumulation of complex sugars. E76.210 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, E76.210 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.

Morquio A is the classic form; ensure you are not confusing it with Morquio B, which has different enzyme deficiency. For E76.210, 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 E76.210 sourced directly from the CMS-HCC risk adjustment model files and the CMS ICD-10-CM code set.

Coding Tips

  • •Morquio A is the classic form; ensure you are not confusing it with Morquio B, which has different enzyme deficiency
  • •Document associated complications such as cardiac valve disease, hearing loss, or vision problems for comprehensive coding

Clinical Significance

Morquio A mucopolysaccharidosis (MPS IVA) is caused by deficiency of N-acetylgalactosamine-6-sulfatase (GALNS), leading to accumulation of keratan sulfate and chondroitin-6-sulfate. It primarily affects the skeleton with severe short stature, spinal cord compression risk, and joint laxity while preserving intelligence, making it distinct from many other MPS types.

Documentation Requirements

  • ✓Specific diagnosis of Morquio A syndrome (MPS IVA) documented
  • ✓Confirmatory enzyme assay showing GALNS deficiency or genetic testing with GALNS gene mutation
  • ✓Skeletal manifestations: short trunk dwarfism, pectus carinatum, genu valgum, odontoid hypoplasia
  • ✓Cervical spine stability assessment (risk of atlantoaxial subluxation/spinal cord compression)
  • ✓Treatment plan including enzyme replacement therapy (elosulfase alfa/Vimizim), surgical interventions, or supportive care

Commonly Confused Codes

  • •E76.211: Morquio B (MPS IVB): different enzyme deficiency (beta-galactosidase), same skeletal phenotype
  • •E76.219: Morquio, unspecified: use only when A vs. B subtype is not documented
  • •E76.1: Hunter syndrome (MPS II): has skeletal features but also hepatosplenomegaly and potential neurodegeneration
  • •Q77.4: Achondroplasia: skeletal dysplasia that may mimic Morquio radiographically but is a different entity
  • •E76.22: Sanfilippo syndrome (MPS III): primarily neurological, not skeletal

Child Codes

E76.210Morquio A mucopolysaccharidoses
HCC 49
E76.211Morquio B mucopolysaccharidoses
HCC 49
E76.219Morquio mucopolysaccharidoses, unspecified
HCC 49

Code Hierarchy

└E76Disorders of glycosaminoglycan metabolism└E76.2Other mucopolysaccharidoses└E76.21Morquio mucopolysaccharidoses└E76.210Morquio A mucopolysaccharidoses
└E76.210Morquio A mucopolysaccharidoses

Also searched as

  • E76 210
  • E76210

For E76.210, 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.

E76.210 maps to CMS-HCC V28 category 49, Specified Lysosomal Storage Disorders. See the ICD-10 to HCC mapping hub for how the V28 crosswalk works. The mapping identifies a payment HCC category for E76.210. Review its source-labeled HCC coefficient above, check the RAF Calculator with complete member context for CMS-HCC V28 PY2026, and confirm the documentation the chart needs before the code is submitted. HCC Buddy shows no RAF score unless every required source and calculation check passes.

Work E76.210 in HCC Buddy

Open E76.210 in the Code Book for the full Index-to-Tabular path, MEAT checklist, and V28 HCC mapping, or in the Encoder to code from a keyword search. Pro includes 7 days to try everything, no card required.

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