E75.240

Niemann-Pick disease type A

Risk-adjusting (HCC)Billable code

HCC risk adjustment mapping

CMS-HCC ESRD V24

HCC23

Other Significant Endocrine and Metabolic Disorders

RxHCC V08

RXHCC41

Lysosomal Storage Disorders

Coding notes

Excludes 1 never coded together · Excludes 2 may co-exist

This code · E75.240

Synonyms

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

Inherited from E75.24Niemann-Pick disease

Synonyms

Acid sphingomyelinase deficiency (ASMD)

Inherited from E75.2Other sphingolipidosis

Excludes 1

adrenoleukodystrophy [Addison-Schilder] (E71.528)

Inherited from E75Disord of sphingolipid metab and oth lipid storage disorders

Excludes 1

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

Inherited from E70-E88Metabolic disorders

Excludes 1

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)

Excludes 2

Ehlers-Danlos syndromes (Q79.6-)

Inherited from chapter 4Endocrine, nutritional and metabolic diseases

Excludes 1

transitory endocrine and metabolic disorders specific to newborn (P70-P74)

Note

All neoplasms, whether functionally active or not, are classified in Chapter 2. Appropriate codes in this chapter (i.e. E05.8, E07.0, E16-E31, E34.-) may be used as additional codes to indicate either functional activity by neoplasms and ectopic endocrine tissue or hyperfunction and hypofunction of endocrine glands associated with neoplasms and other conditions classified elsewhere.

Official Guidelines for E75.240

FY 2026 ›

I.C.4Chapter-specific guidanceChapter 4: Endocrine, Nutritional, and Metabolic Diseases (E00-E89)

Subsections

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