Specific code icon 2014 ICD-10-CM Diagnosis Code E76.01

Hurler's syndrome

  • E76.01 is a billable ICD-10-CM code that can be used to specify a diagnosis.
  • On October 1, 2014 ICD-10-CM will replace ICD-9-CM in the United States, therefore, E76.01 and all other ICD-10-CM codes should only be used for training or planning purposes until then.
Clinical Information
  • 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.
Description Synonyms
  • Mucopolysaccharidosis MPS-I-H
ICD-10-CM E76.01 is grouped within Diagnostic Related Group (MS-DRG v30.0):
  • 642 Inborn and other disorders of metabolism
Convert ICD-10-CM E76.01 to ICD-9-CM
The following ICD-10-CM Index entries contain back-references to ICD-10-CM E76.01: