E75.240 – Niemann-Pick disease type A

ICD-10-CM 2027 diagnosis code · Metabolic disorders

Billable code

ICD-10-CM code E75.240

Code: E75.240 (claims format: E75240)
Description: Niemann-Pick disease type A
Billable: Yes – valid for HIPAA-covered transactions
Valid for: Dates of service October 1, 2026 – September 30, 2027 (FY2027)
Chapter: 4. Endocrine, nutritional and metabolic diseases (E00-E89)
Block: E70-E88 Metabolic disorders
Parent codes: E75 › E75.2 › E75.24

Notes for E75.240

Applicable to:

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

Notes inherited from parent codes

Instructional notes at a category or block level apply to every code below it.

E75 – Disorders of sphingolipid metabolism and other lipid storage disorders

Excludes1 (not coded here):

mucolipidosis, types I-III (E77.0-E77.1)
Refsum's disease (G60.1)

E75.2 – Other sphingolipidosis

Excludes1 (not coded here):

adrenoleukodystrophy [Addison-Schilder] (E71.528)

E75.24 – Niemann-Pick disease

Applicable to:

Acid sphingomyelinase deficiency (ASMD)

Block E70-E88

Excludes1 (not coded here):

androgen insensitivity syndrome (E34.5-)
congenital adrenal hyperplasia (E25.0)
hemolytic anemias attributable to enzyme disorders (D55.-)
Marfan syndrome (Q87.4-)
5-alpha-reductase deficiency (E29.1)

Excludes2 (not included here):

Ehlers-Danlos syndromes (Q79.6-)

Index terms for E75.240

Entries in the ICD-10-CM alphabetic index that lead to this code:

Related codes in E75.24

E75.241 – Niemann-Pick disease type BBillable
E75.242 – Niemann-Pick disease type CBillable
E75.243 – Niemann-Pick disease type DBillable
E75.244 – Niemann-Pick disease type A/BBillable
E75.248 – Other Niemann-Pick diseaseBillable
E75.249 – Niemann-Pick disease, unspecifiedBillable

Frequently asked questions

What is ICD-10 code E75.240?

E75.240 is the ICD-10-CM code for niemann-Pick disease type A, in the block E70-E88 (Metabolic disorders).

Is E75.240 a billable code?

Yes. E75.240 is a billable/specific code that can be used to indicate a diagnosis for reimbursement purposes.

What category does E75.240 belong to?

It belongs to category E75 – Disorders of sphingolipid metabolism and other lipid storage disorders.

Source: CDC/NCHS ICD-10-CM FY2027 code files, tabular list and index. Reference only – code assignment must follow the official ICD-10-CM guidelines and documentation in the medical record.