E75.241

Billable

ChapterE00–E89

Ranksubcategory

Official Description

Niemann-Pick disease type B

Metadata & Insights

•2026 ICD-10-CM Edition - Effective October 1, 2025.
•Billable/specific code (valid for reimbursement).
•Chapter 4: Endocrine, Nutritional and Metabolic Diseases (E00-E89).

Instructional Notes

Includes

Acid sphingomyelinase deficiency type B (ASMD type B)
Chronic visceral acid sphingomyelinase deficiency

General instructions are available for the parent sections:

View CH4 Notes View E70-E88 Notes View E75 Notes View E75.2 Notes View E75.24 Notes

Clinical Hierarchy

CH4Endocrine, nutritional and metabolic diseases (E00-E89)

E70-E88Metabolic disorders (E70-E88)

E75Disorders of sphingolipid metabolism and other lipid storage disorders

E75.2Other sphingolipidosis

E75.24Niemann-Pick disease

E75.241Niemann-Pick disease type B

Applicable Ancestor Context

The following parent sections contain instructions or notes that apply to E75.241:

subcategoryE75.24

Niemann-Pick disease

View context instructions

subcategoryE75.2

Other sphingolipidosis

View context instructions

categoryE75

Disorders of sphingolipid metabolism and other lipid storage disorders

View context instructions

blockE70-E88

Metabolic disorders (E70-E88)

View context instructions

chapterCH4

Endocrine, nutritional and metabolic diseases (E00-E89)

View context instructions

Alphabetic Index Mentions

These are Diagnosis Index lookup paths that route to E75.241. Use them as entry terms for search, then confirm final coding instructions in the Tabular List and notes above.

3 raw paths

Deficiency, deficient2 unique routes

acid sphingomyelinase (ASMD)

type

B→E75.241

chronic visceral acid sphingomyelinase→E75.241

Niemann-Pick disease or syndrome1 unique routes

type

B→E75.241

MS-DRG Classification

2026 v43.1

This code is grouped within the following Diagnosis Related Group(s) for clinical reimbursement:

DRG 642

Inborn and Other Disorders of Metabolism

Cross Reference

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