Home > 2016 ICD-10-CM Diagnosis Codes > Endocrine, nutritional and metabolic diseases E00-E89 > Metabolic disorders E70-E88 > Disorders of glycosaminoglycan metabolism E76-
2016 ICD-10-CM Diagnosis Code E76.01
E76.01 is a specific ICD-10-CM code that can be used to specify a diagnosis.
Reimbursement claims with a date of service on or after October 1, 2015 require the use of ICD-10-CM codes.
This is the American ICD-10-CM version of E76.01. Other international ICD-10 versions may differ.
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.
ICD-10-CM E76.01 is grouped within Diagnostic Related Group (MS-DRG v32.0):