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ICD-10-CM Codes
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E00-E89
Endocrine, nutritional and metabolic diseases
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E70-E88
Metabolic disorders
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E71-
Disorders of branched-chain amino-acid metabolism and fatty-acid metabolism
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2022 ICD-10-CM Diagnosis Code E71.121
2022 ICD-10-CM Diagnosis Code E71.121
Propionic acidemia
2016 2017 2018 2019 2020 2021 2022 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 2022 edition of ICD-10-CM E71.121 became effective on October 1, 2021.
- 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:
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 v39.0):
- 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
- 2022 (effective 10/1/2021): No change
- Acidemia E87.2
ICD-10-CM Diagnosis Code E87.2
Acidosis
2016 2017 2018 2019 2020 2021 2022 Billable/Specific Code
Applicable To- Acidosis NOS
- Lactic acidosis
- Metabolic acidosis
- Respiratory acidosis
Type 1 Excludes- diabetic acidosis - see categories E08-E10, E11, E13 with ketoacidosis
- 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 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 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 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
E71.111 3-methylglutaconic aciduria
E71.118 Other branched-chain organic acidurias
E71.12 Disorders of propionate metabolism
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.