2020 ICD-10-CM Diagnosis Code E71.110

Isovaleric acidemia

    2016 2017 2018 2019 2020 Billable/Specific Code
  • E71.110 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 E71.110 became effective on October 1, 2019.
  • This is the American ICD-10-CM version of E71.110 - other international versions of ICD-10 E71.110 may differ.
The following code(s) above E71.110 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 E71.110:
  • E00-E89
    2020 ICD-10-CM Range E00-E89

    Endocrine, nutritional and metabolic diseases

    • 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
  • Acidemia, isovaleric
  • Isovaleryl-coa dehydrogenase deficiency
Clinical Information
  • A rare autosomal recessive inherited disorder caused by mutations in the ivd gene. It is characterized by abnormalities in the metabolism of leucine. Signs and symptoms vary from very mild to life threatening and include vomiting, seizures, lethargy, and coma.
  • Autosomal recessive aminoacidopathy due to deficiency of isovaleryl-coa dehydrogenase, with elevated plasma isovaleric acid and urinary isovaleric acid and isovalerylglycine, causing a characteristic odor of sweaty feet; clinical signs include severe acidosis and ketosis, lethargy, convulsions, pernicious vomiting, thrombocytopenia, neutropenia, and pancytopenia.
ICD-10-CM E71.110 is grouped within Diagnostic Related Group(s) (MS-DRG v37.0):
  • 642 Inborn and other disorders of metabolism

Convert E71.110 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 E71.110:
  • Acidemia E87.2
    ICD-10-CM Diagnosis Code E87.2


      2016 2017 2018 2019 2020 Billable/Specific Code
    Applicable To
    • Acidosis NOS
    • Lactic acidosis
    • Metabolic acidosis
    • Respiratory acidosis
    Type 1 Excludes
    • diabetic acidosis - see categories E08-E10, E13 with ketoacidosis
    • isovaleric E71.110
  • Disorder (of) - see also Disease
    • metabolism NOS E88.9
      ICD-10-CM Diagnosis Code E88.9

      Metabolic disorder, unspecified

        2016 2017 2018 2019 2020 Billable/Specific Code
      • amino-acid E72.9
        ICD-10-CM Diagnosis Code E72.9

        Disorder of amino-acid metabolism, unspecified

          2016 2017 2018 2019 2020 Billable/Specific Code
        • branched chain E71.2
          ICD-10-CM Diagnosis Code E71.2

          Disorder of branched-chain amino-acid metabolism, unspecified

            2016 2017 2018 2019 2020 Billable/Specific Code
          • isovaleric acidemia E71.110
    • isovaleric acidemia E71.110
  • Isovaleric acidemia E71.110

ICD-10-CM Codes Adjacent To E71.110
E70.40 …… unspecified
E70.41 Histidinemia
E70.49 Other disorders of histidine metabolism
E70.5 Disorders of tryptophan metabolism
E70.8 Other disorders of aromatic amino-acid metabolism
E70.9 Disorder of aromatic amino-acid metabolism, unspecified
E71 Disorders of branched-chain amino-acid metabolism and fatty-acid metabolism
E71.0 Maple-syrup-urine disease
E71.1 Other disorders of branched-chain amino-acid metabolism
E71.11 Branched-chain organic acidurias
E71.110 Isovaleric acidemia
E71.111 3-methylglutaconic aciduria
E71.118 Other branched-chain organic acidurias
E71.12 Disorders of propionate metabolism
E71.120 Methylmalonic acidemia
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

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