E75.240 Niemann-Pick disease type A

The ICD-10-CM code for Niemann-Pick disease type A is E75.240 (FY2026). It is a billable, claim-ready diagnosis code.

Classification

Chapter

4: Endocrine, nutritional and metabolic diseases (E00-E89)

Section

E70-E88: Metabolic disorders (E70-E88)

Category E75

31 codes (26 billable)

FY2026 Status

Stable since FY2024

Also Known As

ICD-10-CM Alphabetic Index entries that lead to E75.240:

• Deficiency, deficient › infantile neurovisceral acid sphingomyelinase
• Niemann-Pick disease or syndrome › type › A
• Deficiency, deficient › acid sphingomyelinase (ASMD) › type › A

Inclusion Terms

• Acid sphingomyelinase deficiency type A (ASMD type A)
• Infantile neurovisceral acid sphingomyelinase deficiency

U.S. Hospital Utilization

E75.240 appeared in too few sampled U.S. inpatient stays in 2023 to report a national estimate (AHRQ suppresses counts of 10 or fewer) — a rare inpatient diagnosis.

Source: National Inpatient Sample (NIS), Healthcare Cost and Utilization Project (HCUP), Agency for Healthcare Research and Quality, 2016–2023. National survey-weighted estimates.

Official Coding Guidelines

Diabetes mellitus (TOC entry)

a. Diabetes mellitus ................................................................................................................... 39

Obesity (TOC entry)

b. Obesity .................................................................................................................................. 42

Source: CMS — ICD-10-CM Official Guidelines for Coding and Reporting, FY2026

References

• CMS — ICD-10-CM Official Code Set and Guidelines, FY2026
• CDC/NCHS — ICD-10-CM Files and Browser Tool
• AHRQ — HCUP National Inpatient Sample, Frequencies by Diagnosis Code (2016–2023)