ICD-10 Code E75.244 – Niemann-Pick disease type A/B (2026): Diagnosis, Symptoms & Billing Guide
2026 ICD-10-CM Diagnosis Code E75.244 – Niemann-Pick disease type A/B
What it is
E75.244 identifies Niemann-Pick disease type A/B, an inherited lysosomal storage disorder caused by acid sphingomyelinase deficiency. Use it when documentation specifies this subtype rather than another Niemann-Pick variant.
Clinical signs
Clinical features vary; refer to documentation. Common findings include hepatosplenomegaly, progressive neurologic involvement, and developmental delay or regression, depending on subtype and disease severity.
When to use this code
Use this code when the provider documents Niemann-Pick disease type A/B, acid sphingomyelinase deficiency, or an equivalent confirmed diagnosis. It may also apply when records clearly identify the A/B phenotype and no more specific code is given. Check documentation if the note only says Niemann-Pick disease without a type.
Do not use for
Do not use it for other Niemann-Pick types, such as type C or unspecified Niemann-Pick disease. Do not assign it when the chart only lists carrier status, family history, or a rule-out diagnosis.
Coding tip
Confirm the exact Niemann-Pick subtype in the assessment, problem list, or genetics report before you code.