2026 ICD-10-CM Diagnosis Code E76.22Sanfilippo mucopolysaccharidoses

ICD-10-CM Codes›E00–E89›E70-E88›E76

ICD-10-CM E76.22
CMSSource: CMS FY 2026 ICD-10-CM dataset · Effective Oct 1, 2025 – Sep 30, 2026

E76.22 is a billable ICD-10-CM diagnosis code for sanfilippo mucopolysaccharidoses. It is valid on HIPAA claims for fiscal year 2026 (October 1, 2025 through September 30, 2026). Coders also document this condition as mucopolysaccharidosis, MPS-III-A. In AHRQ's Clinical Classifications Software (CCSR), this diagnosis falls under Other specified and unspecified nutritional and metabolic disorders.

Code Identity

ICD-10-CM Code
E76.22
Billable Status
Yes — Valid for Submission
Code Describes
Sanfilippo mucopolysaccharidoses
Short Description
Sanfilippo mucopolysaccharidoses
Same as the full description in the CMS dataset.
Parent Code
E76.2
Other mucopolysaccharidoses

Code Classification

ChapterE00–E89Endocrine, nutritional and metabolic diseases
SectionE70-E88Metabolic disorders
CategoryE76Disorders of glycosaminoglycan metabolism
This CodeE76.22Sanfilippo mucopolysaccharidoses

Approximate SynonymsGuidance

Alternate terms and clinical phrases that map to this code.

  • Mucopolysaccharidosis, MPS-III-A
  • Mucopolysaccharidosis, MPS-III-B
  • Mucopolysaccharidosis, MPS-III-C
  • Mucopolysaccharidosis, MPS-III-D
  • Sanfilippo syndrome

Tabular List NotesGuidance

Coding notes and annotation back-references applicable to this code.

Inclusion Terms

  • Mucopolysaccharidosis, type III (A) (B) (C) (D)
  • Sanfilippo A syndrome
  • Sanfilippo B syndrome
  • Sanfilippo C syndrome
  • Sanfilippo D syndrome

Index to Diseases and InjuriesGuidance

Alphabetical index entries that point to this code.

External Cause of Injuries IndexGuidance

References for this code in the External Cause of Injuries Index.

    • Mucopolysaccharidosis
      • Sanfilippo syndrome
    • Mucopolysaccharidosis
      • type
        • III
    • Sanfilippo(Type B) (Type C) (Type D) syndrome

Clinical ClassificationClinical

AHRQ’s CCSR groups this code into broader clinical categories.

CCSR END016
Other specified and unspecified nutritional and metabolic disorders
Default principal diagnosis: inpatient Yes · outpatient Yes

Clinical InformationClinical

  • Mucopolysaccharidoses

    group of lysosomal storage diseases each caused by an inherited deficiency of an enzyme involved in the degradation of glycosaminoglycans (mucopolysaccharides). the diseases are progressive and often display a wide spectrum of clinical severity within one enzyme deficiency.

Patient EducationClinical

Carbohydrate Metabolism Disorders

Metabolism is the process your body uses to make energy from the food you eat. Food is made up of proteins, carbohydrates, and fats. Chemicals in your digestive system (enzymes) break the food parts down into sugars and acids, your body's fuel. Your body can use this fuel right away, or it can store the energy in your body tissues.

Read the full article at MedlinePlus

Courtesy of MedlinePlus, a service of the U.S. National Library of Medicine.

Convert E76.22 to ICD-9-CMHistory

The closest ICD-9-CM equivalents under the General Equivalence Mappings.

ICD-9-CM
277.5 Mucopolysaccharidosis
Approximate The match is approximate rather than exact.

Code HistoryHistory

FY 2016AddedAdded to the ICD-10-CM code setEffective October 1, 2015, the first year of ICD-10-CM.
FY 2017–2025No changes
FY 2026CurrentCurrent code set, no changesEffective October 1, 2025 through September 30, 2026.

Questions About E76.22Overview

Is E76.22 (Other mucopolysaccharidoses) a billable code?

Yes. This is a billable ICD-10-CM code, specific enough to report sanfilippo mucopolysaccharidoses on HIPAA-covered claims from October 1, 2025 through September 30, 2026.

What is the ICD-9 equivalent of E76.22?

Under the General Equivalence Mappings, sanfilippo mucopolysaccharidoses converts to ICD-9-CM 277.5 (mucopolysaccharidosis). The mapping is approximate, so confirm the match fits the documentation.

Footnotes

[1] Chronic - a chronic condition code indicates a condition lasting 12 months or longer and its effect on the patient based on one or both of the following criteria:

  • The condition results in the need for ongoing intervention with medical products,treatment, services, and special equipment
  • The condition places limitations on self-care, independent living, and social interactions.