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E75.244 ICD-10-CM Code: Niemann-Pick disease type A/B

E75.244 is not a CMS-HCC payment code. MEAT criteria · RAF Calculator · free HCC coding tools

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Code lookupE75.244

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

E75.244

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

Niemann-Pick disease type A/B

Niemann-Pick disease type A/B is a combined form of the inherited metabolic disorder showing characteristics of both type A and type B presentations with variable severity and progression.

Buddy the Bee presenting code insight

Buddy Insight

Niemann-Pick disease type A/B is an intermediate phenotype of acid sphingomyelinase deficiency showing features of both the severe neuronopathic type A and the milder non-neuronopathic type B.

OverviewRisk ModelsCode BookDocumentationAuditNotesEdition comparison

CMS-HCC V28

N/A

—

Not mapped

CMS-HCC V24

Historical

Historical

Not used for CY2026 payment

ACA/HHS

HCC 027

Code-level coefficient reference

ESRD/PACE

HCC 23

Code-level coefficient reference

RXHCC

HCC 41

Code-level coefficient reference

Code Book Path

Official
E75.2Other sphingolipidosisE75.24Niemann-Pick diseaseE75.244Niemann-Pick disease type A/B

Inclusion Terms

Official
  • Acid sphingomyelinase deficiency type A/B (ASMD type A/B)
  • Chronic neurovisceral acid sphingomyelinase deficiency
  • Acid sphingomyelinase deficiency (ASMD)Inherited from E75.24

Excludes 2

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

Related Codes

Official
E75.240Niemann-Pick disease type AE75.241Niemann-Pick disease type BE75.242Niemann-Pick disease type CE75.243Niemann-Pick disease type DE75.248Other Niemann-Pick disease

Includes

Official

No Includes notes are included in this display for E75.244. 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, E75.2
  • androgen insensitivity syndrome (E34.5-)Inherited from E00-E89, E70-E88, E75, E75.2
  • congenital adrenal hyperplasia (E25.0)Inherited from E00-E89, E70-E88, E75, E75.2
  • hemolytic anemias attributable to enzyme disorders (D55.-)Inherited from E00-E89, E70-E88, E75, E75.2
  • Marfan syndrome (Q87.4-)Inherited from E00-E89, E70-E88, E75, E75.2
  • 5-alpha-reductase deficiency (E29.1)Inherited from E00-E89, E70-E88, E75, E75.2
  • mucolipidosis, types I-III (E77.0-E77.1)Inherited from E00-E89, E70-E88, E75, E75.2
  • Refsum's disease (G60.1)Inherited from E00-E89, E70-E88, E75, E75.2
  • adrenoleukodystrophy [Addison-Schilder] (E71.528)Inherited from E00-E89, E70-E88, E75, E75.2

Code First

Official

No Code First sequencing instructions are included in this display for E75.244. Check the code and parent instructions in the Code Book.

Use Additional

Official

No Use Additional Code instructions are included in this display for E75.244. Check the code and parent instructions in the Code Book.

Code Also

Official

No Code Also instructions are included in this display for E75.244. Check the code and parent instructions in the Code Book.

Buddy Documentation Tip

HCC Buddy guidance
Confirmed diagnosis of Niemann-Pick disease type A/B or intermediate acid sphingomyelinase deficiency
Acid sphingomyelinase enzyme assay results showing intermediate deficiency
SMPD1 gene mutation analysis with documentation of variant type
Assessment of both neurological and visceral manifestations

MEAT Support

HCC Buddy guidance
Confirmed diagnosis of Niemann-Pick disease type A/B or intermediate acid sphingomyelinase deficiency
Acid sphingomyelinase enzyme assay results showing intermediate deficiency
SMPD1 gene mutation analysis with documentation of variant type
Assessment of both neurological and visceral manifestations

Audit Caution

HCC Buddy guidance
Forcing the diagnosis into either type A or type B when the intermediate phenotype A/B has its own code
Confusing the A/B intermediate with type C, which is a completely different disease
Using the unspecified code when the intermediate phenotype is clearly documented
Not coding both the neurological and visceral manifestations as additional diagnoses

Common Mistakes

HCC Buddy guidance
E75.240 — Niemann-Pick disease type A: severe neuronopathic form with early fatality
E75.241 — Niemann-Pick disease type B: non-neuronopathic visceral form
E75.242 — Niemann-Pick disease type C: different gene and enzyme pathway
E75.249 — Niemann-Pick disease, unspecified: do not use when type A/B is documented

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 E75.244 an HCC code?

E75.244 has no mapping under the current CMS-HCC V28 community payment model. E75.244 has a separate mapping under the CMS-HCC ESRD model (HCC 23 (Other Significant Endocrine and Metabolic Disorders)) and the Part D RxHCC model (HCC 41 (Lysosomal Storage Disorders)); the applicable result needs member context. E75.244 also appears in the HHS-HCC commercial risk model (HCC 027 (HHS-HCC 027 adult, RAF varies by metal level)), which is a commercial market model rather than a Medicare Advantage payment mapping.

Code
E75.244
Description
Niemann-Pick disease type A/B
HCC (V28)
No CMS-HCC V28 mapping
RAF reference coefficient
—
Billable
Yes
Payment year
2026

HCC Category Mapping

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 E75.244 in the Code Book — tabular path, V28 RAF reference, and MEAT checklist →

MEAT review for E75.244

For E75.244, 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

E75.244 is the ICD-10-CM diagnosis code for niemann-pick disease type a/b. Niemann-Pick disease type A/B is a combined form of the inherited metabolic disorder showing characteristics of both type A and type B presentations with variable severity and progression. E75.244 sits in the ICD-10-CM chapter for endocrine, nutritional and metabolic diseases (e00-e89), within the section covering metabolic disorders (e70-e88).

E75.244 has no mapping under the current CMS-HCC V28 community payment model. E75.244 has a separate mapping under the CMS-HCC ESRD model (HCC 23 (Other Significant Endocrine and Metabolic Disorders)) and the Part D RxHCC model (HCC 41 (Lysosomal Storage Disorders)); the applicable result needs member context. E75.244 also appears in the HHS-HCC commercial risk model (HCC 027 (HHS-HCC 027 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.

This code indicates overlap features of both types; ensure documentation clearly describes which characteristics are present.

HCC Buddy maintains structured V28 and V24 mapping, source-labeled coefficient references, and MEAT documentation criteria for E75.244 sourced directly from the CMS-HCC risk adjustment model files and the CMS ICD-10-CM code set.

Coding Tips

  • •This code indicates overlap features of both types; ensure documentation clearly describes which characteristics are present
  • •Code specific organ involvement and neurological manifestations separately based on clinical presentation

Clinical Significance

Niemann-Pick disease type A/B is an intermediate phenotype of acid sphingomyelinase deficiency showing features of both the severe neuronopathic type A and the milder non-neuronopathic type B. Patients may have some neurological involvement along with hepatosplenomegaly and lung disease. The clinical course is variable, and enzyme replacement therapy (olipudase alfa) may be applicable for the visceral manifestations.

Documentation Requirements

  • ✓Confirmed diagnosis of Niemann-Pick disease type A/B or intermediate acid sphingomyelinase deficiency
  • ✓Acid sphingomyelinase enzyme assay results showing intermediate deficiency
  • ✓SMPD1 gene mutation analysis with documentation of variant type
  • ✓Assessment of both neurological and visceral manifestations
  • ✓Hepatosplenomegaly documentation with imaging
  • ✓Current treatment plan and monitoring schedule

Commonly Confused Codes

  • •E75.240: Niemann-Pick disease type A: severe neuronopathic form with early fatality
  • •E75.241: Niemann-Pick disease type B: non-neuronopathic visceral form
  • •E75.242: Niemann-Pick disease type C: different gene and enzyme pathway
  • •E75.249: Niemann-Pick disease, unspecified: do not use when type A/B is documented
  • •E75.248: Other Niemann-Pick disease: for variants not fitting standard types

Child Codes

E75.240Niemann-Pick disease type A
E75.241Niemann-Pick disease type B
E75.242Niemann-Pick disease type C
E75.243Niemann-Pick disease type D
E75.244Niemann-Pick disease type A/B
E75.248Other Niemann-Pick disease
E75.249Niemann-Pick disease, unspecified

Code Hierarchy

└E75Disorders of sphingolipid metabolism and other lipid storage disorders└E75.2Other sphingolipidosis└E75.24Niemann-Pick disease└E75.244Niemann-Pick disease type A/B
└E75.244Niemann-Pick disease type A/B

E75.244 code history

Code setChange
FY2022 (effective Oct 1, 2021)Added to the code set

Source: official CMS ICD-10-CM order and addenda files, FY2016 through FY2027.

Also searched as

  • E75 244
  • E75244

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