1. ICD-10-CM Codes
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2. E00-E89 Endocrine, nutritional and metabolic diseases
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3. E70-E88 Metabolic disorders
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4. E71- Disorders of branched-chain amino-acid metabolism and fatty-acid metabolism
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5. 2021 ICD-10-CM Diagnosis Code E71.121

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.

• 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.

• 642 Inborn and other disorders of metabolism

Convert E71.121 to ICD-9-CM

• 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

• 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

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