ICD-10 Code E75.240 – Niemann-Pick disease type A (2026): Diagnosis, Symptoms & Billing Guide
2026 ICD-10-CM Diagnosis Code E75.240 – Niemann-Pick disease type A
What it is
E75.240 identifies Niemann-Pick disease type A, a rare inherited lysosomal storage disorder. It reflects a severe sphingolipid metabolism defect that causes harmful material to build up in cells.
Clinical signs
Typical findings include early-onset neurodegeneration, developmental delay, hypotonia, and progressive loss of skills. Hepatosplenomegaly and failure to thrive are common; clinical features vary, so refer to documentation.
When to use this code
Use this code when the provider documents Niemann-Pick disease type A specifically, or when the chart confirms the type A form through genetic or specialist evaluation. It may also apply when the diagnosis is established and recorded in the problem list. Check documentation if the type is not clearly stated.
Do not use for
Do not use E75.240 for other Niemann-Pick types, such as type B or mixed/unspecified forms. If the record only says “Niemann-Pick disease” without a type, check documentation.
Coding tip
Code the exact subtype documented; Niemann-Pick type A should not be assumed from a general lysosomal storage disorder diagnosis.