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E76.219 ICD-10-CM Code: Morquio mucopolysaccharidoses, unspecified

E76.219 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 lookupE76.219

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

E76.219

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

Morquio mucopolysaccharidoses, unspecified

Morquio mucopolysaccharidosis, unspecified type, is a rare inherited metabolic disorder causing skeletal deformities and progressive physical problems when the specific type (A or B) is not documented.

Buddy the Bee presenting code insight

Buddy Insight

Morquio mucopolysaccharidoses, unspecified is used when MPS IV is diagnosed but the subtype (A vs.

OverviewRisk ModelsCode BookDocumentationAuditNotesEdition comparison

CMS-HCC V28

HCC 49

Code-level coefficient reference

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 mucopolysaccharidosesE76.219Morquio mucopolysaccharidoses, unspecified

Inclusion Terms

Official
  • Morquio syndrome
  • Mucopolysaccharidosis, type IV

Excludes 2

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

Related Codes

Official
E76.210Morquio A mucopolysaccharidosesE76.211Morquio B mucopolysaccharidoses

Includes

Official

No Includes notes are included in this display for E76.219. 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 E76.219. Check the code and parent instructions in the Code Book.

Use Additional

Official

No Use Additional Code instructions are included in this display for E76.219. Check the code and parent instructions in the Code Book.

Code Also

Official

No Code Also instructions are included in this display for E76.219. Check the code and parent instructions in the Code Book.

Buddy Documentation Tip

HCC Buddy guidance
Provider diagnosis of Morquio syndrome or MPS IV without specification of A or B subtype
Clinical findings consistent with MPS IV: skeletal dysplasia, short trunk dwarfism, joint laxity, corneal clouding
Reason subtype is not specified (testing pending, unavailable, or not yet ordered)
Current symptom management and care plan

MEAT Support

HCC Buddy guidance
Provider diagnosis of Morquio syndrome or MPS IV without specification of A or B subtype
Clinical findings consistent with MPS IV: skeletal dysplasia, short trunk dwarfism, joint laxity, corneal clouding
Reason subtype is not specified (testing pending, unavailable, or not yet ordered)
Current symptom management and care plan

Audit Caution

HCC Buddy guidance
Using this unspecified code when the medical record contains enzyme results or genetic testing that would specify A or B
Not querying the provider for subtype clarification when enzyme or genetic data is available
Confusing this with E76.3 (MPS unspecified) — E76.219 indicates Morquio IS identified, just not the subtype
Failing to code cervical spine instability risk which is critical in all Morquio patients

Common Mistakes

HCC Buddy guidance
E76.210 — Morquio A: use when GALNS deficiency is confirmed
E76.211 — Morquio B: use when beta-galactosidase deficiency is confirmed
E76.3 — Mucopolysaccharidosis, unspecified: even broader; use only when the MPS type is entirely unknown
E76.29 — Other mucopolysaccharidoses: for non-Morquio MPS types that lack specific codes

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 E76.219 an HCC code?

Yes. E76.219 (Morquio mucopolysaccharidoses, unspecified) 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: E76.219 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
E76.219
Description
Morquio mucopolysaccharidoses, unspecified
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 E76.219 in the Code Book — tabular path, V28 RAF reference, and MEAT checklist →

MEAT review for E76.219

For E76.219, 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

E76.219 is the ICD-10-CM diagnosis code for morquio mucopolysaccharidoses, unspecified. Morquio mucopolysaccharidosis, unspecified type, is a rare inherited metabolic disorder causing skeletal deformities and progressive physical problems when the specific type (A or B) is not documented. E76.219 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, E76.219 maps to Specified Lysosomal Storage Disorders (HCC 49) with a source-labeled community, non-dual, aged reference coefficient of 9.256. No V24 mapping is shown for E76.219; use the applicable model and payment year when reviewing the V28 mapping. 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.

Use this code only when the specific Morquio type (A or B) cannot be determined from the medical record. For E76.219, 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 E76.219 sourced directly from the CMS-HCC risk adjustment model files and the CMS ICD-10-CM code set.

Coding Tips

  • •Use this code only when the specific Morquio type (A or B) cannot be determined from the medical record
  • •Query the provider to determine the specific type if possible, as this affects treatment options and genetic counseling

Clinical Significance

Morquio mucopolysaccharidoses, unspecified is used when MPS IV is diagnosed but the subtype (A vs. B) has not been determined or documented. This code indicates a need for further diagnostic workup since treatment options differ between subtypes, particularly with elosulfase alfa being specific to Morquio A.

Documentation Requirements

  • ✓Provider diagnosis of Morquio syndrome or MPS IV without specification of A or B subtype
  • ✓Clinical findings consistent with MPS IV: skeletal dysplasia, short trunk dwarfism, joint laxity, corneal clouding
  • ✓Reason subtype is not specified (testing pending, unavailable, or not yet ordered)
  • ✓Current symptom management and care plan
  • ✓Referral for enzyme assay or genetic testing if not yet performed

Commonly Confused Codes

  • •E76.210: Morquio A: use when GALNS deficiency is confirmed
  • •E76.211: Morquio B: use when beta-galactosidase deficiency is confirmed
  • •E76.3: Mucopolysaccharidosis, unspecified: even broader; use only when the MPS type is entirely unknown
  • •E76.29: Other mucopolysaccharidoses: for non-Morquio MPS types that lack specific codes

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└E76.219Morquio mucopolysaccharidoses, unspecified
└E76.219Morquio mucopolysaccharidoses, unspecified

For E76.219, 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.

E76.219 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 E76.219. 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 E76.219 in HCC Buddy

Open E76.219 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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