Disorders of glycosaminoglycan metabolism (E76) ICD-10-CM
The E76 code range covers disorders of glycosaminoglycan metabolism with 16 ICD-10-CM diagnosis codes. 12 of them are billable and valid for claim submission in fiscal year 2026, and the category headers group them but cannot themselves be billed.
Codes in the E76 Range 16 codes · 12 billable
- E76 Disorders of glycosaminoglycan metabolismNon-billable
- E76.0 Mucopolysaccharidosis, type INon-billable
- E76.01 Hurler's syndrome
- E76.02 Hurler-Scheie syndrome
- E76.03 Scheie's syndrome
- E76.1 Mucopolysaccharidosis, type II
- E76.2 Other mucopolysaccharidosesNon-billable
- E76.21 Morquio mucopolysaccharidosesNon-billable
- E76.210 Morquio A mucopolysaccharidoses
- E76.211 Morquio B mucopolysaccharidoses
- E76.219 Morquio mucopolysaccharidoses, unspecified
- E76.22 Sanfilippo mucopolysaccharidoses
- E76.29 Other mucopolysaccharidoses
- E76.3 Mucopolysaccharidosis, unspecified
- E76.8 Other disorders of glucosaminoglycan metabolism
- E76.9 Glucosaminoglycan metabolism disorder, unspecified
Clinical Terms in This Code Range
Definitions from the National Library of Medicine for conditions coded in the E76 range.
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.
About the E76 Code Range
The ICD-10 code section E76 covers disorders of glycosaminoglycan metabolism, including various types of mucopolysaccharidoses (MPS). These conditions are rare inherited metabolic diseases characterized by enzyme deficiencies affecting the breakdown of glycosaminoglycans.
Codes like E76.0 through E76.3 specify different mucopolysaccharidosis types. For example, E76.01 refers to Hurler's syndrome, also known as MPS-I-H or dysostosis multiplex, while E76.1 represents MPS-II, commonly called Hunter's syndrome. The section further categorizes variants including Morquio syndromes (codes E76.210 and E76.211) and Sanfilippo syndrome (E76.22). Specific distinctions among these codes help medical coders accurately identify the exact disorder, such as differentiating between Morquio A and B forms. Unspecified or other mucopolysaccharidoses and glucosaminoglycan metabolism disorders are captured under codes like E76.3 and E76.9. Using these codes correctly supports precise documentation and treatment planning for these complex metabolic diseases.
Questions About This Page
How many billable codes are in the E76 range?
Of the 16 codes in this range, 12 are billable and valid for claim submission from October 1, 2025 through September 30, 2026. Category header codes group them but cannot be reported on claims.
What does the E76 range classify?
The range classifies disorders of glycosaminoglycan metabolism. Each code links to its own reference page with billing status, MS-DRG grouping, coding notes, and clinical information.
Related References
Source: CMS FY 2026 ICD-10-CM Tabular List and order file, effective October 1, 2025 through September 30, 2026.
