E76.22 ICD-10-CM Code: Sanfilippo mucopolysaccharidoses
A clear reference for the official code description, hierarchy, and related coding information from the FY 2027 U.S. release.
Official description
E76.22Sanfilippo mucopolysaccharidoses
ICD-10-CM diagnosis code · United States clinical modification
Where E76.22 sits
- 1
Chapter 4
Endocrine, nutritional and metabolic diseases (E00-E89)
- 2
Section
Metabolic disorders (E70-E88)
- 3
Category E76
Disorders of glycosaminoglycan metabolism
- 4
Subcategory E76.2
Other mucopolysaccharidoses
- 5
Code E76.22
Sanfilippo mucopolysaccharidoses
Notes for this code
Inclusion terms
- Mucopolysaccharidosis, type III (A) (B) (C) (D)
- Sanfilippo A syndrome
- Sanfilippo B syndrome
- Sanfilippo C syndrome
- Sanfilippo D syndrome
Code first
No “code first” instruction is listed for E76.22.
Use additional code
No “use additional code” instruction is listed for E76.22.
Excludes1
Conditions that should not be reported together with this code.
No Excludes1 note is listed for E76.22.
Excludes2
Conditions not included here that may be reported separately when present.
No Excludes2 note is listed for E76.22.
Source and release
- Data release
- U.S. ICD-10-CM · FY 2027
- Effective date
- Oct 1, 2026
- Publisher
- Vero Scribe Inc.
This reference is generated from Vero’s ICD-10-CM description, hierarchy, and code-note dataset. The visible content and structured data share that source.
Frequently asked questions
What does ICD-10-CM code E76.22 mean?
E76.22 is the FY 2027 U.S. ICD-10-CM code for “Sanfilippo mucopolysaccharidoses.”
Is E76.22 a billable or specific ICD-10-CM code?
Yes. In the FY 2027 reference data used on this page, E76.22 has no child codes and is presented as a billable/specific code. Always confirm the active code set and payer requirements before use.
Where is E76.22 located in ICD-10-CM?
E76.22 is listed in Chapter 4, Endocrine, nutritional and metabolic diseases (E00-E89), within Metabolic disorders (E70-E88) (E70–E88), under category E76, Disorders of glycosaminoglycan metabolism and subcategory E76.2, Other mucopolysaccharidoses.
What coding notes are listed for E76.22?
Its listed inclusion terms are Mucopolysaccharidosis, type III (A) (B) (C) (D); Sanfilippo A syndrome; Sanfilippo B syndrome; Sanfilippo C syndrome; Sanfilippo D syndrome. No code-first instruction is listed. No Excludes1 note is listed. No Excludes2 note is listed.