Home > 2014 ICD-10-CM Diagnosis Codes > Endocrine, nutritional and metabolic diseases E00-E89 > Metabolic disorders E70-E88 > Disorders of glycosaminoglycan metabolism E76-
2014 ICD-10-CM Diagnosis Code E76.01
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.
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 v30.0):