2021 ICD-10-CM Diagnosis Code E71.121

Propionic acidemia

    2016 2017 2018 2019 2020 2021 Billable/Specific Code
  • E71.121 is a billable/specific ICD-10-CM code that can be used to indicate a diagnosis for reimbursement purposes.
  • The 2021 edition of ICD-10-CM E71.121 became effective on October 1, 2020.
  • This is the American ICD-10-CM version of E71.121 - other international versions of ICD-10 E71.121 may differ.
The following code(s) above E71.121 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 E71.121:
  • E00-E89
    2021 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
    2021 ICD-10-CM Range E70-E88

    Metabolic disorders

    Type 1 Excludes
    • androgen insensitivity syndrome (E34.5-)
    • congenital adrenal hyperplasia (E25.0)
    • Ehlers-Danlos syndromes (Q79.6-)
    • hemolytic anemias attributable to enzyme disorders (D55.-)
    • Marfan's syndrome (Q87.4)
    • 5-alpha-reductase deficiency (E29.1)
    Metabolic disorders
Clinical Information
  • A rare autosomal inherited organic acid disorder caused by mutations in the pcca and pccb genes. It results in the accumulation of harmful organic acids in the blood and urine. Signs and symptoms appear in infancy and include vomiting, poor feeding, loss of appetite, hypotonia, and lethargy.
  • Autosomal recessive metabolic disorder caused by mutations in propionyl-coa carboxylase genes that result in dysfunction of branch chain amino acids and of the metabolism of certain fatty acids. Neonatal clinical onset is characterized by severe metabolic acidemia accompanied by hyperammonemia, hyperglycemia, lethargy, vomiting, hypotonia; and hepatomegaly. Survivors of the neonatal onset propionic acidemia often show developmental retardation, and intolerance to dietary proteins. Late-onset form of the disease shows mild mental and/or developmental retardation, sometimes without metabolic acidemia.
  • Elevated accumulation and excretion of glycine accompanied by ketosis that is secondary to methylmalonicacidemia, isovalericacidemia and other organic acidemias, as distinguished from nonketotic hyperglycinemia.
ICD-10-CM E71.121 is grouped within Diagnostic Related Group(s) (MS-DRG v38.0):
  • 642 Inborn and other disorders of metabolism

Convert E71.121 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

Diagnosis Index entries containing back-references to E71.121:
  • Acidemia E87.2
    ICD-10-CM Diagnosis Code E87.2

    Acidosis

      2016 2017 2018 2019 2020 2021 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
    • propionic E71.121
  • 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 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 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 Billable/Specific Code
          • proprionic acidemia E71.121
    • propionic acidemia E71.121
  • Propionic acidemia E71.121

ICD-10-CM Codes Adjacent To E71.121
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
E71.313 Glutaric aciduria type II

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