ICD-10 Code E76.0 – Mucopolysaccharidosis, type I (2026): Diagnosis, Symptoms & Billing Guide
2026 ICD-10-CM Diagnosis Code E76.0 – Mucopolysaccharidosis, type I
What it is
E76.0 identifies mucopolysaccharidosis type I, a lysosomal storage disorder caused by deficient breakdown of glycosaminoglycans. It is a congenital metabolic condition that leads to progressive multisystem disease.
Clinical signs
Clinical features vary; refer to documentation. Commonly reported findings include developmental delay, coarse facial features, skeletal abnormalities, hepatosplenomegaly, and corneal clouding.
When to use this code
Use E76.0 when the record clearly documents mucopolysaccharidosis type I, including Hurler, Hurler-Scheie, or Scheie phenotype if specified by the provider. You may also use it when the diagnosis is confirmed and the note does not require a more specific subtype code. Check documentation if the chart only mentions a suspected inherited storage disorder or an unspecified mucopolysaccharidosis.
Do not use for
Do not use E76.0 for other mucopolysaccharidoses, such as types II, III, IV, VI, or VII. Do not assign it when the condition is only being ruled out or when documentation supports a different metabolic disorder.
Coding tip
Confirm the exact mucopolysaccharidosis type in the assessment before coding, because subtype wording in the chart determines whether E76.0 is appropriate.