E76.01
Hurler’s syndrome
Approximate Synonyms
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Mucopolysaccharidosis, mps-i-h
Clinical Information
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An autosomal recessive inherited disorder of mucopolysaccharide metabolism. It is the most severe form of mucopolysaccharidosis type i. It is characterized by deficiency of the enzyme alpha-l-iduronidase resulting in the accumulation of mucopolysaccharides in the tissues.
Details
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E76.01 is a billable/specific ICD-10-CM code that can be used to indicate a diagnosis for reimbursement purposes.
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The 2023 edition of ICD-10-CM E76.01 became effective on October 1, 2022.
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This is the American ICD-10-CM version of E76.01 – other international versions of ICD-10 E76.01 may differ.