2020 ICD-10-CM Diagnosis Code E76.1

Mucopolysaccharidosis, type II

    2016 2017 2018 2019 2020 Billable/Specific Code
  • E76.1 is a billable/specific ICD-10-CM code that can be used to indicate a diagnosis for reimbursement purposes.
  • The 2020 edition of ICD-10-CM E76.1 became effective on October 1, 2019.
  • This is the American ICD-10-CM version of E76.1 - other international versions of ICD-10 E76.1 may differ.
Applicable To
  • Hunter's syndrome
The following code(s) above E76.1 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 E76.1:
  • E00-E89
    2020 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
    2020 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 2
  • Mucopolysaccharidosis, mps-ii
Clinical Information
  • A syndrome with variable manifestations exhibiting mainly microcephaly, characteristic facies, mental retardation, short stature, acral skeletal anomalies with occasional craniosynostosis and congenital heart defects.
  • An inborn mucopolysaccharide metabolism disorder with iduronate-2-sulfatase deficiency. Clinical characteristics are similar to those in mps i, except for the absence of corneal clouding and slower progression of the course of disease and central nervous system deterioration. Retinal degeneration may occur, but the cornea usually remains clear. Appearance is normal at birth with excessive growth taking place during first two years of life. Two types are recognized: a severe form (mps iia) which is characterized mainly by mental retardation and progressive physical deterioration and early death, and a mild form (mps iib) in which patients may survive into adulthood. Mps iia usually occurs between 2 and 4 years of age with progressive deterioration, chronic diarrhea, recurrent ear infections, hearing impairment, communicating hydrocephalus with increased intracranial pressure, and death at about 10 and 15 years. Obstructive airway disease, cardiac valvular dysfunction, myocardial thickening, pulmonary hypertension, coronary disease, and myocardial infarction may be superimposed. Mps iib is milder with preservation of intelligence. The symptoms usually include hearing impairment, carpal tunnel syndrome, joint stiffness, discrete corneal opacities, and papilledema. Death may occur in early adulthood, usually from airway obstruction or cardiac failure.
  • An x-linked, inherited lysosomal storage disease caused by the deficiency of the enzyme iduronate sulfatase that is responsible for the degradation of mucopolysaccharides. It nearly always affects males and is characterized by the accumulation of mucopolysaccharides in various organs, resulting in mental dysfunction, enlarged abdomen, hearing loss, obstructive airway disease, heart disease, and hepatosplenomegaly.
  • Lysosomal storage disease marked by progressive physical deterioration and caused by a deficiency of l-sulfoiduronate sulfatase; this disease differs from muchopolysaccharidosis i by slower progression, lack of corneal clouding, and x-linked rather than autosomal recessive inheritance.
  • Systemic lysosomal storage disease marked by progressive physical deterioration and caused by a deficiency of l-sulfoiduronate sulfatase. This disease differs from mucopolysaccharidosis i by slower progression, lack of corneal clouding, and x-linked rather than autosomal recessive inheritance. The mild form produces near-normal intelligence and life span. The severe form usually causes death by age 15.
ICD-10-CM E76.1 is grouped within Diagnostic Related Group(s) (MS-DRG v37.0):
  • 642 Inborn and other disorders of metabolism

Convert E76.1 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
  • 2019 (effective 10/1/2018): No change
  • 2020 (effective 10/1/2019): No change

Diagnosis Index entries containing back-references to E76.1:
  • Degeneration, degenerative
    • brain (cortical) (progressive) G31.9
      ICD-10-CM Diagnosis Code G31.9

      Degenerative disease of nervous system, unspecified

        2016 2017 2018 2019 2020 Billable/Specific Code
      • in
        • Hunter's syndrome E76.1
  • Hunter's
    • syndrome E76.1
  • Mucopolysaccharidosis E76.3
    ICD-10-CM Diagnosis Code E76.3

    Mucopolysaccharidosis, unspecified

      2016 2017 2018 2019 2020 Billable/Specific Code
    • type
      • II E76.1
    • Hunter's syndrome E76.1

ICD-10-CM Codes Adjacent To E76.1
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
E76.3 Mucopolysaccharidosis, unspecified
E76.8 Other disorders of glucosaminoglycan metabolism
E76.9 Glucosaminoglycan metabolism disorder, unspecified

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