2019 ICD-10-CM Diagnosis Code E76.3

Mucopolysaccharidosis, unspecified

    2016 2017 2018 2019 Billable/Specific Code
  • E76.3 is a billable/specific ICD-10-CM code that can be used to indicate a diagnosis for reimbursement purposes.
  • The 2019 edition of ICD-10-CM E76.3 became effective on October 1, 2018.
  • This is the American ICD-10-CM version of E76.3 - other international versions of ICD-10 E76.3 may differ.
The following code(s) above E76.3 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 E76.3:
  • E00-E89
    2019 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
    2019 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
Clinical Information
  • A group of autosomal recessive or x-linked inherited lysosomal storage disorders affecting the metabolism of mucopolysaccharides, resulting in the accumulation of mucopolysaccharides in the body. Signs and symptoms include organomegaly, mental retardation, abnormal skeletal development, heart disorders, hearing loss, and central nervous system deficiencies.
  • Any of a group of lysosomal storage diseases resulting from defects in degradation of glycosaminoglycans, which are excreted in urine and accumulate in tissue.
  • Group of lysosomal storage diseases each caused by an inherited deficiency of an enzyme involved in the degradation of glycosaminoglycans (mucopolysaccharides). The diseases are progressive and often display a wide spectrum of clinical severity within one enzyme deficiency.
ICD-10-CM E76.3 is grouped within Diagnostic Related Group(s) (MS-DRG v36.0):
  • 642 Inborn and other disorders of metabolism

Convert E76.3 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
Code annotations containing back-references to E76.3:
  • Code First: I52
    ICD-10-CM Diagnosis Code I52

    Other heart disorders in diseases classified elsewhere

      2016 2017 2018 2019 Billable/Specific Code Manifestation Code
    Code First
    • underlying disease, such as:
    • congenital syphilis (A50.5)
    • mucopolysaccharidosis (E76.3)
    • schistosomiasis (B65.0-B65.9)
    Type 1 Excludes
    • heart disease (in):
    • gonococcal infection (A54.83)
    • meningococcal infection (A39.50)
    • rheumatoid arthritis (M05.31)
    • syphilis (A52.06)
  • Type 1 Excludes: Q77
    , E74
    ICD-10-CM Diagnosis Code Q77

    Osteochondrodysplasia with defects of growth of tubular bones and spine

      2016 2017 2018 2019 Non-Billable/Non-Specific Code
    Type 1 Excludes
    Type 2 Excludes
    • congenital myotonic chondrodystrophy (G71.13)
    ICD-10-CM Diagnosis Code E74

    Other disorders of carbohydrate metabolism

      2016 2017 2018 2019 Non-Billable/Non-Specific Code
    Type 1 Excludes

Diagnosis Index entries containing back-references to E76.3:
  • Cardiopathy I51.9
    - see also Disease, heart
    ICD-10-CM Diagnosis Code I51.9

    Heart disease, unspecified

      2016 2017 2018 2019 Billable/Specific Code
    • mucopolysaccharidosis E76.3
  • Mucopolysaccharidosis E76.3
    • cardiopathy E76.3

ICD-10-CM Codes Adjacent To E76.3
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
E77 Disorders of glycoprotein metabolism
E77.0 Defects in post-translational modification of lysosomal enzymes
E77.1 Defects in glycoprotein degradation
E77.8 Other disorders of glycoprotein metabolism
E77.9 Disorder of glycoprotein metabolism, unspecified
E78 Disorders of lipoprotein metabolism and other lipidemias
E78.0 Pure hypercholesterolemia
E78.00 …… unspecified

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