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E75.19 ICD-10-CM Code: Other gangliosidosis

E75.19 is not a CMS-HCC payment code. MEAT criteria · RAF Calculator · free HCC coding tools

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Code lookupE75.19

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

E75.19

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

Other gangliosidosis

A rare inherited metabolic disorder where fatty substances (gangliosides) accumulate in cells, causing neurological problems, with a specific type not listed in other gangliosidosis categories.

Buddy the Bee presenting code insight

Buddy Insight

Other gangliosidosis captures ganglioside storage disorders not classified as GM2 gangliosidosis or mucolipidosis IV, most notably GM1 gangliosidosis (beta-galactosidase deficiency).

CMS-HCC V28

N/A

Not mapped

CMS-HCC V24

Historical

Historical

Not used for CY2026 payment

ACA/HHS

HCC 119

Code-level coefficient reference

ESRD/PACE

HCC 52

Code-level coefficient reference

RXHCC

HCC 41

Code-level coefficient reference

Inclusion Terms

Official
  • GM1 gangliosidosis
  • GM3 gangliosidosis

Excludes 2

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

Includes

Official

No Includes notes are included in this display for E75.19. 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, E75
  • androgen insensitivity syndrome (E34.5-)Inherited from E00-E89, E70-E88, E75
  • congenital adrenal hyperplasia (E25.0)Inherited from E00-E89, E70-E88, E75
  • hemolytic anemias attributable to enzyme disorders (D55.-)Inherited from E00-E89, E70-E88, E75
  • Marfan syndrome (Q87.4-)Inherited from E00-E89, E70-E88, E75
  • 5-alpha-reductase deficiency (E29.1)Inherited from E00-E89, E70-E88, E75
  • mucolipidosis, types I-III (E77.0-E77.1)Inherited from E00-E89, E70-E88, E75
  • Refsum's disease (G60.1)Inherited from E00-E89, E70-E88, E75

Code First

Official

No Code First sequencing instructions are included in this display for E75.19. Check the code and parent instructions in the Code Book.

Use Additional

Official

No Use Additional Code instructions are included in this display for E75.19. Check the code and parent instructions in the Code Book.

Code Also

Official

No Code Also instructions are included in this display for E75.19. Check the code and parent instructions in the Code Book.

Buddy Documentation Tip

HCC Buddy guidance
Specific gangliosidosis variant documented (e.g., GM1 gangliosidosis with type specification)
Enzyme assay results (beta-galactosidase for GM1) or genetic testing
Clinical form specified: infantile, late infantile/juvenile, or adult/chronic
Documentation of skeletal, neurological, and visceral manifestations

MEAT Support

HCC Buddy guidance
Specific gangliosidosis variant documented (e.g., GM1 gangliosidosis with type specification)
Enzyme assay results (beta-galactosidase for GM1) or genetic testing
Clinical form specified: infantile, late infantile/juvenile, or adult/chronic
Documentation of skeletal, neurological, and visceral manifestations

Audit Caution

HCC Buddy guidance
Coding GM1 gangliosidosis under the GM2 gangliosidosis codes (E75.00-E75.09) instead of E75.19
Using the unspecified gangliosidosis code when the specific type is documented
Confusing GM1 gangliosidosis with mucopolysaccharidoses due to overlapping skeletal features
Not coding skeletal, hepatic, and neurological manifestations as additional diagnoses

Common Mistakes

HCC Buddy guidance
E75.10 — Unspecified gangliosidosis: use only when no specific type can be identified
E75.11 — Mucolipidosis IV: separate specific code for ML IV
E75.00-E75.09 — GM2 gangliosidosis: for GM2 variants specifically
E76.01-E76.02 — Mucopolysaccharidosis Type I: can have overlapping skeletal features with GM1

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 E75.19 an HCC code?

E75.19 is not in the CMS-HCC V28 or V24 community payment model. E75.19 has a separate mapping under the CMS-HCC ESRD model (HCC 52 (Dementia Without Complication)) and the Part D RxHCC model (HCC 41 (Lysosomal Storage Disorders)); the applicable result needs member context. E75.19 also appears in the HHS-HCC commercial risk model (HCC 119 (HHS-HCC 119 adult, RAF varies by metal level)), which is a commercial market model rather than a Medicare Advantage payment mapping.

Code
E75.19
Description
Other gangliosidosis
HCC (V28)
No CMS-HCC V28 mapping
RAF reference coefficient
Billable
Yes
Payment year
2026

HCC Category Mapping

ESRDHCC 52, Dementia Without Complication
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 E75.19 in the Code Book — tabular path, V28 RAF reference, and MEAT checklist →

MEAT review for E75.19

For E75.19, 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

E75.19 is the ICD-10-CM diagnosis code for other gangliosidosis. A rare inherited metabolic disorder where fatty substances (gangliosides) accumulate in cells, causing neurological problems, with a specific type not listed in other gangliosidosis categories. E75.19 sits in the ICD-10-CM chapter for endocrine, nutritional and metabolic diseases (e00-e89), within the section covering metabolic disorders (e70-e88).

E75.19 has no mapping under the CMS-HCC V28 or V24 community payment models. E75.19 has a separate mapping under the CMS-HCC ESRD model (HCC 52 (Dementia Without Complication)) and the Part D RxHCC model (HCC 41 (Lysosomal Storage Disorders)); the applicable result needs member context. E75.19 also appears in the HHS-HCC commercial risk model (HCC 119 (HHS-HCC 119 adult, RAF varies by metal level)), which is a commercial market model rather than a Medicare Advantage payment mapping. Do not assign V28 risk adjustment value from this page; verify the applicable model and payment year before using this code for risk adjustment.

Use this code only when the gangliosidosis type is documented but doesn't fit E75.11 or other specific codes.

HCC Buddy maintains structured V28 and V24 mapping, source-labeled coefficient references, and MEAT documentation criteria for E75.19 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 gangliosidosis type is documented but doesn't fit E75.11 or other specific codes
  • Verify genetic testing documentation supports the specific gangliosidosis diagnosis

Clinical Significance

Other gangliosidosis captures ganglioside storage disorders not classified as GM2 gangliosidosis or mucolipidosis IV, most notably GM1 gangliosidosis (beta-galactosidase deficiency). GM1 gangliosidosis causes progressive neurodegeneration, skeletal abnormalities, and hepatosplenomegaly. Accurate coding of the specific gangliosidosis type is important for genetic counseling and disease management.

Documentation Requirements

  • Specific gangliosidosis variant documented (e.g., GM1 gangliosidosis with type specification)
  • Enzyme assay results (beta-galactosidase for GM1) or genetic testing
  • Clinical form specified: infantile, late infantile/juvenile, or adult/chronic
  • Documentation of skeletal, neurological, and visceral manifestations
  • Family history and genetic counseling documentation

Commonly Confused Codes

  • E75.10: Unspecified gangliosidosis: use only when no specific type can be identified
  • E75.11: Mucolipidosis IV: separate specific code for ML IV
  • E75.00-E75.09: GM2 gangliosidosis: for GM2 variants specifically
  • E76.01-E76.02: Mucopolysaccharidosis Type I: can have overlapping skeletal features with GM1
  • E75.22: Gaucher disease: different lysosomal storage disorder

Child Codes

Code Hierarchy

Also searched as

  • E75 19
  • E7519

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