2026 ICD-10-CM Diagnosis Code E71.121Propionic acidemia

ICD-10-CM Codes›E00–E89›E70-E88›E71

ICD-10-CM E71.121
CMSSource: CMS FY 2026 ICD-10-CM dataset · Effective Oct 1, 2025 – Sep 30, 2026

E71.121 is a billable ICD-10-CM diagnosis code for propionic acidemia. It is valid on HIPAA claims for fiscal year 2026 (October 1, 2025 through September 30, 2026). Coders also document this condition as deficiency of propionyl-CoA carboxylase. In AHRQ's Clinical Classifications Software (CCSR), this diagnosis falls under Other specified and unspecified nutritional and metabolic disorders.

Code Identity

ICD-10-CM Code
E71.121
Billable Status
Yes — Valid for Submission
Code Describes
Propionic acidemia
Short Description
Propionic acidemia
Same as the full description in the CMS dataset.
Parent Code
E71.12
Disorders of propionate metabolism

Code Classification

ChapterE00–E89Endocrine, nutritional and metabolic diseases
SectionE70-E88Metabolic disorders
CategoryE71Disorders of branched-chain amino-acid metabolism and fatty-acid metabolism
This CodeE71.121Propionic acidemia

Approximate SynonymsGuidance

Alternate terms and clinical phrases that map to this code.

  • Deficiency of propionyl-CoA carboxylase
  • Propionic acidemia
  • Propionic acidemia, type I
  • Propionic acidemia, type II
  • Propionyl-CoA carboxylase deficiency pccA complementation group
  • Propionyl-CoA carboxylase deficiency pccBC complementation group

Index to Diseases and InjuriesGuidance

Alphabetical index entries that point to this code.

External Cause of Injuries IndexGuidance

References for this code in the External Cause of Injuries Index.

    • Acidemia
      • propionic
    • Disorder(of)
      • metabolism NOS
        • amino-acid
          • branched chain
            • propionic acidemia
    • Disorder(of)
      • propionic acidemia
    • Propionic acidemia

Clinical ClassificationClinical

AHRQ’s CCSR groups this code into broader clinical categories.

CCSR END016
Other specified and unspecified nutritional and metabolic disorders
Default principal diagnosis: inpatient Yes · outpatient Yes

Clinical InformationClinical

  • Propionic Acidemia

    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.

  • Propionic Acidemia

    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.

Patient EducationClinical

Metabolic Disorders

Metabolism is the process your body uses to get or make energy from the food you eat. Food is made up of proteins, carbohydrates, and fats. Chemicals in your digestive system break the food parts down into sugars and acids, your body's fuel.

Read the full article at MedlinePlus

Courtesy of MedlinePlus, a service of the U.S. National Library of Medicine.

Convert E71.121 to ICD-9-CMHistory

The closest ICD-9-CM equivalents under the General Equivalence Mappings.

ICD-9-CM
270.3 Bran-chain amin-acid dis
Approximate The match is approximate rather than exact.

Code HistoryHistory

FY 2016AddedAdded to the ICD-10-CM code setEffective October 1, 2015, the first year of ICD-10-CM.
FY 2017–2025No changes
FY 2026CurrentCurrent code set, no changesEffective October 1, 2025 through September 30, 2026.

Questions About E71.121Overview

Is E71.121 (Disorders of propionate metabolism) a billable code?

Yes. This is a billable ICD-10-CM code, specific enough to report propionic acidemia on HIPAA-covered claims from October 1, 2025 through September 30, 2026.

What is the ICD-9 equivalent of E71.121?

Under the General Equivalence Mappings, propionic acidemia converts to ICD-9-CM 270.3 (bran-chain amin-acid dis). The mapping is approximate, so confirm the match fits the documentation.

Footnotes

[1] Chronic - a chronic condition code indicates a condition lasting 12 months or longer and its effect on the patient based on one or both of the following criteria:

  • The condition results in the need for ongoing intervention with medical products,treatment, services, and special equipment
  • The condition places limitations on self-care, independent living, and social interactions.