2020 ICD-10-CM Diagnosis Code E71.313

Glutaric aciduria type II

    2016 2017 2018 2019 2020 Billable/Specific Code
  • E71.313 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 E71.313 became effective on October 1, 2019.
  • This is the American ICD-10-CM version of E71.313 - other international versions of ICD-10 E71.313 may differ.
Applicable To
  • Glutaric aciduria type II A
  • Glutaric aciduria type II B
  • Glutaric aciduria type II C
Type 1 Excludes
Type 1 Excludes Help
A type 1 excludes note is a pure excludes. It means "not coded here". A type 1 excludes note indicates that the code excluded should never be used at the same time as E71.313. A type 1 excludes note is for used for when two conditions cannot occur together, such as a congenital form versus an acquired form of the same condition.
  • glutaric aciduria (type 1) NOS (
    ICD-10-CM Diagnosis Code E72.3

    Disorders of lysine and hydroxylysine metabolism

      2016 2017 2018 2019 2020 Billable/Specific Code
    Applicable To
    • Glutaric aciduria NOS
    • Glutaric aciduria (type I)
    • Hydroxylysinemia
    • Hyperlysinemia
    Type 1 Excludes
    E72.3
    )
The following code(s) above E71.313 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 E71.313:
  • 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
  • E71.3
    ICD-10-CM Diagnosis Code E71.3

    Disorders of fatty-acid metabolism

      2016 2017 2018 2019 2020 Non-Billable/Non-Specific Code
    Type 1 Excludes
    • peroxisomal disorders (E71.5)
    • Refsum's disease (G60.1)
    • Schilder's disease (G37.0)
    Type 2 Excludes
    • carnitine deficiency due to inborn error of metabolism (E71.42)
    Disorders of fatty-acid metabolism
Approximate Synonyms
  • Fatty acid oxidation disorder, madd
  • Glutaric aciduria, type 2
Clinical Information
  • A rare autosomal recessive inherited metabolic disorder caused by mutations in the etfa, etfb, and etfdh genes. It is characterized by defects of the electron transfer flavoprotein or electron transfer flavoprotein dehydrogenase. Clinical presentations include congenital abnormalities, hepatomegaly, metabolic acidosis, and hypotonia.
  • A rare autosomal recessive inherited metabolic disorder caused by mutations in the etfa, etfb, or etfdh genes. It is characterized by deficiency of the enzyme electron transfer flavoprotein or the enzyme electron transfer flavoprotein dehydrogenase. It results in metabolic acidosis, hypoglycemia, and behavioral changes. Additional abnormalities include brain malformations, facial malformations, dilated cardiomyopathy, and hepatomegaly.
  • An autosomal recessive disorder of fatty acid oxidation, and branched chain amino acids (amino acids, branched-chain); lysine; and choline catabolism, that is due to defects in either subunit of electron transfer flavoprotein or its dehydrogenase, electron transfer flavoprotein-ubiquinone oxidoreductase (ec 1.5.5.1).
ICD-10-CM E71.313 is grouped within Diagnostic Related Group(s) (MS-DRG v37.0):
  • 642 Inborn and other disorders of metabolism

Convert E71.313 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
Code annotations containing back-references to E71.313:
  • Type 1 Excludes: E72.3
    ICD-10-CM Diagnosis Code E72.3

    Disorders of lysine and hydroxylysine metabolism

      2016 2017 2018 2019 2020 Billable/Specific Code
    Applicable To
    • Glutaric aciduria NOS
    • Glutaric aciduria (type I)
    • Hydroxylysinemia
    • Hyperlysinemia
    Type 1 Excludes

Diagnosis Index entries containing back-references to E71.313:
  • Aciduria
    • glutaric (type I) E72.3
      ICD-10-CM Diagnosis Code E72.3

      Disorders of lysine and hydroxylysine metabolism

        2016 2017 2018 2019 2020 Billable/Specific Code
      Applicable To
      • Glutaric aciduria NOS
      • Glutaric aciduria (type I)
      • Hydroxylysinemia
      • Hyperlysinemia
      Type 1 Excludes
      • type II E71.313

ICD-10-CM Codes Adjacent To E71.313
E71.121 Propionic acidemia
E71.128 Other disorders of propionate metabolism
E71.19 Other disorders of branched-chain amino-acid metabolism
E71.2 Disorder of branched-chain amino-acid metabolism, unspecified
E71.3 Disorders of fatty-acid metabolism
E71.30 Disorder of fatty-acid metabolism, unspecified
E71.31 Disorders of fatty-acid oxidation
E71.310 Long chain/very long chain acyl CoA dehydrogenase deficiency
E71.311 Medium chain acyl CoA dehydrogenase deficiency
E71.312 Short chain acyl CoA dehydrogenase deficiency
E71.313 Glutaric aciduria type II
E71.314 Muscle carnitine palmitoyltransferase deficiency
E71.318 Other disorders of fatty-acid oxidation
E71.32 Disorders of ketone metabolism
E71.39 Other disorders of fatty-acid metabolism
E71.4 Disorders of carnitine metabolism
E71.40 Disorder of carnitine metabolism, unspecified
E71.41 Primary carnitine deficiency
E71.42 Carnitine deficiency due to inborn errors of metabolism
E71.43 Iatrogenic carnitine deficiency
E71.44 Other secondary carnitine deficiency

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