ICD-10 Code E76 – Disorders of glycosaminoglycan metabolism (2026): Diagnosis, Symptoms & Billing Guide
2026 ICD-10-CM Diagnosis Code E76 – Disorders of glycosaminoglycan metabolism
What it is
E76 identifies inherited disorders that affect the breakdown of glycosaminoglycans, leading to mucopolysaccharide storage disease. These conditions cause buildup of complex sugars in tissues and organs.
Clinical signs
Clinical features vary; refer to documentation. Common findings include coarse facial features, skeletal abnormalities, developmental delay, organ enlargement, and progressive multisystem involvement.
When to use this code
Use E76 when the record documents a diagnosis of a glycosaminoglycan metabolism disorder or mucopolysaccharidosis without a more specific subtype. You may also use it when the clinician states the condition broadly and does not name the exact syndrome. Check documentation for the specific inherited disorder if available.
Do not use for
Do not use E76 for unrelated metabolic disorders or for nonspecific symptoms without a documented diagnosis. If the chart names a specific mucopolysaccharidosis type, code that more specific condition instead.
Coding tip
Review the full note for the exact mucopolysaccharidosis subtype before assigning the unspecified category.