Skip to content
Dx

ICD-10-CM 2027 diagnosis code

E75.244Niemann-Pick disease type A/B

✓ Billable / specific

E75.244 is a valid, billable ICD-10-CM code for niemann-pick disease type a/b. It can be reported on claims for dates of service from October 1, 2026 – September 30, 2027.

Code last changed in FY2022 (effective October 1, 2021). Source: official FY2027 ICD-10-CM release from CDC/NCHS and CMS. Page updated September 29, 2026. About our data

About E75.244

E75.244 is the ICD-10-CM diagnosis code for Niemann-Pick disease type A/B. It belongs to category E75 (disorders of sphingolipid metabolism and other lipid storage disorders), block E70-E88 (metabolic disorders) and chapter 4 (endocrine, nutritional and metabolic diseases). It is billable, so it can be reported on its own for dates of service from October 1, 2026 through September 30, 2027.

Within E75.24 (Niemann-Pick disease), E75.244 is specifically for Niemann-Pick disease type A/B. Related codes cover type A (E75.240), type B (E75.241), type C (E75.242), type D (E75.243), other Niemann-Pick disease (E75.248) and unspecified (E75.249).

It is not a CC or MCC, so as a secondary diagnosis it doesn't change the MS-DRG severity level.

In MS-DRG v44.0, it is part of the grouping logic for DRG 642 (Inborn and Other Disorders of Metabolism, relative weight 1.3219), in MDC 10 (Endocrine, Nutritional and Metabolic Diseases and Disorders), as a principal or secondary diagnosis. The DRG a claim lands in depends on the principal diagnosis, procedures and any CC/MCC secondary diagnoses.

It was added in FY2022, effective October 1, 2021.

Coding notes

Applicable toConditions and synonyms classified to this code
  • Acid sphingomyelinase deficiency type A/B (ASMD type A/B)
  • Chronic neurovisceral acid sphingomyelinase deficiency

Notes that apply from higher levels

Instructions written at a parent level also apply to E75.244.

› From E75.2 Other sphingolipidosis
Excludes1
  • adrenoleukodystrophy [Addison-Schilder] (E71.528)
› From E75 Disorders of sphingolipid metabolism and other lipid storage disorders
Excludes1

Broader instructions also apply from E70-E88 Metabolic disorders and Chapter 4: Endocrine, nutritional and metabolic diseases.

Alphabetic index entries

3 entries

Terms in the official ICD-10-CM index that lead to E75.244.

MS-DRG v44.0 grouping

All DRGs →

Inpatient MS-DRGs this diagnosis can group to, as the principal or a secondary diagnosis, depending on the rest of the claim.

MDC 10 · Endocrine, Nutritional and Metabolic Diseases and Disorders

Code history

  1. 2016
  2. 2017
  3. 2018
  4. 2019
  5. 2020
  6. 2021
  7. 2022
  8. 2023
  9. 2024
  10. 2025
  11. 2026
  12. 2027
  • FY2022 (effective 10/1/2021): Added
  • No changes since FY2022.

Common questions about E75.244

Is E75.244 billable?

Yes. E75.244 is a billable/specific ICD-10-CM code valid for FY2027 (October 1, 2026 – September 30, 2027).

Can E75.244 be used as a principal diagnosis?

Yes. The Medicare Code Editor doesn't restrict E75.244 as a principal diagnosis.

Is E75.244 a CC or MCC?

No. E75.244 is neither a CC nor an MCC under MS-DRG v44.0.

What DRG does E75.244 group to?

E75.244 is used in the MS-DRG v44.0 logic for MS-DRG 642 (Inborn and Other Disorders of Metabolism). Which one applies depends on whether it is the principal diagnosis and on the rest of the claim.