2026 ICD-10-CM Diagnosis Code E75.244Niemann-Pick disease type A/B

ICD-10-CM Codes›E00–E89›E70-E88›E75

ICD-10-CM E75.244
CMSSource: CMS FY 2026 ICD-10-CM dataset · Effective Oct 1, 2025 – Sep 30, 2026

E75.244 is a billable ICD-10-CM diagnosis code for Niemann-Pick disease type A/B. It is valid on HIPAA claims for fiscal year 2026 (October 1, 2025 through September 30, 2026). Coders also document this condition as chronic neurovisceral acid sphingomyelinase deficiency. In AHRQ's Clinical Classifications Software (CCSR), this diagnosis falls under Other specified and unspecified nutritional and metabolic disorders.

Code Identity

ICD-10-CM Code
E75.244
Billable Status
Yes — Valid for Submission
Code Describes
Niemann-Pick disease type A/B
Short Description
Niemann-Pick disease type A/B
Same as the full description in the CMS dataset.
Parent Code
E75.24
Niemann-Pick disease

Code Classification

ChapterE00–E89Endocrine, nutritional and metabolic diseases
SectionE70-E88Metabolic disorders
CategoryE75Disorders of sphingolipid metabolism and other lipid storage disorders
This CodeE75.244Niemann-Pick disease type A/B

Approximate SynonymsGuidance

Alternate terms and clinical phrases that map to this code.

  • Chronic neurovisceral acid sphingomyelinase deficiency
  • Lipoidosis
  • Sphingomyelin/cholesterol lipidosis

Tabular List NotesGuidance

Coding notes and annotation back-references applicable to this code.

Inclusion Terms

  • Acid sphingomyelinase deficiency type A/B (ASMD type A/B)
  • Chronic neurovisceral acid sphingomyelinase deficiency

Index to Diseases and InjuriesGuidance

Alphabetical index entries that point to this code.

External Cause of Injuries IndexGuidance

References for this code in the External Cause of Injuries Index.

    • Deficiency, deficient
      • acid sphingomyelinase (ASMD)
        • type
          • A/B
    • Deficiency, deficient
      • chronic neurovisceral acid sphingomyelinase
    • Niemann-Pick disease or syndrome
      • type
        • A/B

Clinical ClassificationClinical

AHRQ’s CCSR groups this code into broader clinical categories.

CCSR END016
Other specified and unspecified nutritional and metabolic disorders
Default principal diagnosis: inpatient Yes · outpatient Yes

Patient EducationClinical

Genetic Brain Disorders

A genetic brain disorder is caused by a variation or a mutation in a gene. A variation is a different form of a gene. A mutation is a change in a gene. Genetic brain disorders affect the development and function of the brain.

Read the full article at MedlinePlus

Courtesy of MedlinePlus, a service of the U.S. National Library of Medicine.

Code History & ChangesHistory

Replacement E75.244 replaces the following previously assigned code(s):

  • E75.240 - Niemann-Pick disease type A
  • E75.241 - Niemann-Pick disease type B
  • E75.248 - Other Niemann-Pick disease
  • E75.249 - Niemann-Pick disease, unspecified
FY 2022AddedAdded to the ICD-10-CM code setEffective October 1, 2021.
FY 2023–2025No changes
FY 2026CurrentCurrent code set, no changesEffective October 1, 2025 through September 30, 2026.

Questions About E75.244Overview

Is E75.244 (Niemann-Pick disease) a billable code?

Yes. This is a billable ICD-10-CM code, specific enough to report Niemann-Pick disease type A/B on HIPAA-covered claims from October 1, 2025 through September 30, 2026.

Footnotes

[1] Chronic - a chronic condition code indicates a condition lasting 12 months or longer and its effect on the patient based on one or both of the following criteria:

  • The condition results in the need for ongoing intervention with medical products,treatment, services, and special equipment
  • The condition places limitations on self-care, independent living, and social interactions.