E75.240 Niemann-Pick disease type A

Billable ICD-10-CM code, FY2026.

Applicable to

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

Applies from E75 Disorders of sphingolipid metabolism and other lipid storage disorders

Excludes1 (never code together)

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

Applies from E75.2 Other sphingolipidosis

Excludes1 (never code together)

• adrenoleukodystrophy [Addison-Schilder] (E71.528)

Applies from E75.24 Niemann-Pick disease

Applicable to

• Acid sphingomyelinase deficiency (ASMD)

Risk adjustment (HCC)

• CMS-HCC V22: HCC 23 — Other Significant Endocrine and Metabolic Disorders
• RxHCC V08: HCC 41 — Lysosomal Storage Disorders
• ESRD V24: HCC 23

MS-DRG index

Defines the logic of DRG(s) 642 in MDC 10.

Approximate ICD-9-CM (GEMs)

• 272.7 Lipidoses (approximate)

Code annotations containing back-references to E75.240

Back-references to E75.2

• Type 1 Excludes: E78 Disorders of lipoprotein metabolism and other lipidemias ("sphingolipidosis (E75.0-E75.3)")

Back-references to E75

• Type 2 Excludes: G11 Hereditary ataxia ("metabolic disorders (E70-E88)")
• Code First: G63 Polyneuropathy in diseases classified elsewhere ("metabolic diseases (E70-E88)")
• Type 2 Excludes: G71 Primary disorders of muscles ("metabolic disorders (E70-E88)")
• Code First: G73.7 Myopathy in diseases classified elsewhere ("lipid storage disorders (E75.-)")
• Code First: H36 Retinal disorders in diseases classified elsewhere ("lipid storage disorders (E75.-)")
• Code First: H42 Glaucoma in diseases classified elsewhere ("specified metabolic disorder (E70-E88)")
• Type 2 Excludes: N25.0 Renal osteodystrophy ("metabolic disorders classifiable to E70-E88")
• Type 2 Excludes: N25.81 Secondary hyperparathyroidism of renal origin ("metabolic disorders classifiable to E70-E88")
• Type 1 Excludes: P59 Neonatal jaundice from other and unspecified causes ("jaundice due to inborn errors of metabolism (E70-E88)")
• Type 1 Excludes: R79 Other abnormal findings of blood chemistry ("specific findings indicating disorder of lipid metabolism (E75.-)")

Diagnosis Index entries for E75.240

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

Nearby codes

• E75.2 Other sphingolipidosis
• E75.21 Fabry (-Anderson) disease
• E75.22 Gaucher disease
• E75.23 Krabbe disease
• E75.24 Niemann-Pick disease
• E75.240 Niemann-Pick disease type A (this code)
• E75.241 Niemann-Pick disease type B
• E75.242 Niemann-Pick disease type C
• E75.243 Niemann-Pick disease type D
• E75.244 Niemann-Pick disease type A/B
• E75.248 Other Niemann-Pick disease