2023 ICD-10-CM Diagnosis Code E71.120

Methylmalonic acidemia

    2016 2017 2018 2019 2020 2021 2022 2023 Billable/Specific Code
  • E71.120 is a billable/specific ICD-10-CM code that can be used to indicate a diagnosis for reimbursement purposes.
  • The 2023 edition of ICD-10-CM E71.120 became effective on October 1, 2022.
  • This is the American ICD-10-CM version of E71.120 - other international versions of ICD-10 E71.120 may differ.
The following code(s) above E71.120 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 E71.120:
  • E00-E89
    2023 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
    2023 ICD-10-CM Range E70-E88

    Metabolic disorders

    Type 1 Excludes
    • androgen insensitivity syndrome (E34.5-)
    • congenital adrenal hyperplasia (E25.0)
    • hemolytic anemias attributable to enzyme disorders (D55.-)
    • Marfan's syndrome (Q87.4)
    • 5-alpha-reductase deficiency (E29.1)
    Type 2 Excludes
    • Ehlers-Danlos syndromes (Q79.6-)
    Metabolic disorders
Approximate Synonyms
  • Cobalamin c disease
  • Methylmalonic acidemia, cobalamin c type
Clinical Information
  • A rare autosomal recessive inherited disorder caused by mutations of the mut, mmaa, mmab, mmadhc, and mcee genes. It is characterized by abnormalities in the metabolism of lipids and proteins. Signs and symptoms usually appear early in life and vary from mild to life threatening. They include vomiting, dehydration, hypotonia, developmental delays, hepatomegaly, lethargy, intellectual disabilities, and chronic kidney disease.
  • Autosomal recessive aminoacidopathy characterized by an excess of methylmalonic acid in the blood and urine, with metabolic ketoacidosis, hyperglycinemia, hyperglycinuria and hyperammonemia; results from defects that cause deficiencies of methylmalonyl-coa mutase.
ICD-10-CM E71.120 is grouped within Diagnostic Related Group(s) (MS-DRG v40.0):
  • 642 Inborn and other disorders of metabolism

Convert E71.120 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
  • 2021 (effective 10/1/2020): No change
  • 2022 (effective 10/1/2021): No change
  • 2023 (effective 10/1/2022): No change
Code annotations containing back-references to E71.120:
  • Code Also: D59.32
    ICD-10-CM Diagnosis Code D59.32

    Hereditary hemolytic-uremic syndrome

      2023 - New Code Billable/Specific Code
    Applicable To
    • Atypical hemolytic uremic syndrome with an identified genetic cause
    Code Also
    • , if applicable:
    • defects in the complement system (D84.1)
    • methylmalonic acidemia (E71.120)

Diagnosis Index entries containing back-references to E71.120:
  • Acidemia E87.20
    - see also Acidosis
    ICD-10-CM Diagnosis Code E87.20

    Acidosis, unspecified

      2023 - New Code Billable/Specific Code
    Applicable To
    • Lactic acidosis NOS
    • Metabolic acidosis NOS
    Code Also
    • , if applicable, respiratory failure with hypercapnia (J96. with 5th character 2)
    • methylmalonic E71.120
  • Disorder (of) - see also Disease
    • metabolism NOS E88.9
      ICD-10-CM Diagnosis Code E88.9

      Metabolic disorder, unspecified

        2016 2017 2018 2019 2020 2021 2022 2023 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 2021 2022 2023 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 2021 2022 2023 Billable/Specific Code
          • methylmalonic acidemia E71.120
    • methylmalonic acidemia E71.120
  • Methylmalonic acidemia E71.120

ICD-10-CM Codes Adjacent To E71.120
E70.89 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
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

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