E71.121 Propionic acidemia

ICD-10-CM Code E71.121: Propionic Acidemia

ICD-10-CM code E71.121 represents Propionic acidemia, an active, billable diagnosis classified under disorders of propionate metabolism. The condition is an intoxication-type organic acidemia involving nonfunctional propionyl CoA carboxylase. This reference synthesizes official terminology records and clinical excerpts to explain the code and its clinical context. 1 2 4 5

Plain-language overview

Propionic acidemia is an intoxication-type disorder of organic metabolism that can present in the neonatal period with lethargy, poor feeding, and vomiting, and may progress to coma if not identified and treated appropriately. 2

The condition involves a nonfunctional propionyl CoA carboxylase that affects the conversion of amino acids and fats into sugar for energy, and it is also known as ketotic hyperglycinemia or propionyl-CoA carboxylase deficiency. 4

What this code represents

ICD-10-CM code E71.121 is the specific billable diagnosis code for Propionic acidemia, valid for reimbursement, with an active record status in the 2026 version of the terminology. 1 5

The semantic type assigned to code E71.121 is Disease or Syndrome, and the concept has narrower concepts within the ICD-10-CM terminology structure, indicating further specification exists. 1

Coding details

Code E71.121 is a child of the parent concept E71.12, which encompasses Disorders of propionate metabolism in the ICD-10-CM coding hierarchy, establishing its categorical placement. 1

Documentation considerations

Clinical documentation for symptomatic patients may include laboratory testing, physical exam findings, and history, as these elements are evaluated during metabolic decompensation in individuals with propionic acidemia. 2

A quick diagnostic pathway for propionic acidemia is used to evaluate a sick neonate or a neonate with an elevated propionylcarnitine on newborn screening, which may inform clinical documentation. 2

What the sources add

While the clinical literature outlines acute management and dietary interventions for propionic acidemia, the official ICD-10-CM terminology record establishes the code's hierarchical structure and active status without detailing treatment protocols. 1 2

The disease ontology reference maps E71.121 to multiple terminology systems including SNOMED CT and UMLS, whereas the official ICD-10-CM tool confirms the code label, providing distinct operational context for coding professionals. 3 4

Sources

1. ICD-10-CM E71.121 terminology record — National Cancer Institute Enterprise Vocabulary Services; accessed 2026-07-27.
2. Acute Management of Propionic Acidemia — pmc.ncbi.nlm.nih.gov; accessed 2026-07-27.
3. ICD-10-CM — icd10cmtool.cdc.gov; accessed 2026-07-27.
4. None — disease-ontology.org; accessed 2026-07-27.
5. E71.121 — Propionic acidemia | ICD-10 Code · Eleplan — eleplan.com; accessed 2026-07-27.