ICD-10-CM Codes
E00-E89 Endocrine, nutritional and metabolic diseases
E70-E88 Metabolic disorders
E76- Disorders of glycosaminoglycan metabolism

2018 ICD-10-CM Diagnosis Code E76.01

Hurler's syndrome

2016

2017

2018

Billable/Specific Code

E76.01 is a billable/specific ICD-10-CM code that can be used to indicate a diagnosis for reimbursement purposes.
The 2018 edition of ICD-10-CM E76.01 became effective on October 1, 2017.
This is the American ICD-10-CM version of E76.01 - other international versions of ICD-10 E76.01 may differ.

The following code(s) above E76.01 contain annotation back-references

Annotation Back-References

In this context, annotation back-references refer to codes that contain:

Applicable To annotations, or
Code Also annotations, or
Code First annotations, or
Excludes1 annotations, or
Excludes2 annotations, or
Includes annotations, or
Note annotations, or
Use Additional annotations

that may be applicable to E76.01:

E00-E89
2018 ICD-10-CM Range E00-E89
Endocrine, nutritional and metabolic diseases
Note
- All neoplasms, whether functionally active or not, are classified in Chapter 2. Appropriate codes in this chapter (i.e. E05.8, E07.0, E16-E31, E34.-) may be used as additional codes to indicate either functional activity by neoplasms and ectopic endocrine tissue or hyperfunction and hypofunction of endocrine glands associated with neoplasms and other conditions classified elsewhere.
Type 1 Excludes
- transitory endocrine and metabolic disorders specific to newborn (P70-P74)
Endocrine, nutritional and metabolic diseases
E70-E88
2018 ICD-10-CM Range E70-E88
Metabolic disorders
Type 1 Excludes
- androgen insensitivity syndrome (E34.5-)
- congenital adrenal hyperplasia (E25.0)
- Ehlers-Danlos syndrome (Q79.6)
- hemolytic anemias attributable to enzyme disorders (D55.-)
- Marfan's syndrome (Q87.4)
- 5-alpha-reductase deficiency (E29.1)
Metabolic disorders

Approximate Synonyms

Mucopolysaccharidosis, mps-i-h

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.

ICD-10-CM E76.01 is grouped within Diagnostic Related Group(s) (MS-DRG v35.0):

642 Inborn and other disorders of metabolism

Convert E76.01 to ICD-9-CM

Code History

2016 (effective 10/1/2015): New code (first year of non-draft ICD-10-CM)
2017 (effective 10/1/2016): No change
2018 (effective 10/1/2017): No change

Diagnosis Index entries containing back-references to E76.01:

Dysostosis
- multiplex E76.01
Gargoylism E76.01
Lipochondrodystrophy E76.01
Mucopolysaccharidosis E76.3
ICD-10-CM Diagnosis Code E76.3
Mucopolysaccharidosis, unspecified
- type
  - I
    - Hurler's syndrome E76.01
- Hurler's syndrome E76.01

ICD-10-CM Codes Adjacent To E76.01

E75.248 Other Niemann-Pick disease

E75.249 …… unspecified

E75.25 Metachromatic leukodystrophy

E75.29 Other sphingolipidosis

E75.3 Sphingolipidosis, unspecified

E75.4 Neuronal ceroid lipofuscinosis

E75.5 Other lipid storage disorders

E75.6 Lipid storage disorder, unspecified

E76 Disorders of glycosaminoglycan metabolism

E76.0 Mucopolysaccharidosis, type I

E76.01 Hurler's syndrome

E76.02 Hurler-Scheie syndrome

E76.03 Scheie's syndrome

E76.1 Mucopolysaccharidosis, type II

E76.2 Other mucopolysaccharidoses

E76.21 Morquio mucopolysaccharidoses

E76.210 Morquio A mucopolysaccharidoses

E76.211 Morquio B mucopolysaccharidoses

E76.219 …… unspecified

E76.22 Sanfilippo mucopolysaccharidoses

E76.29 Other mucopolysaccharidoses

Reimbursement claims with a date of service on or after October 1, 2015 require the use of ICD-10-CM codes.

2018 ICD-10-CM Diagnosis Code E76.01

Hurler's syndrome

Endocrine, nutritional and metabolic diseases

Metabolic disorders

Mucopolysaccharidosis, unspecified