ICD-10 Code E71.121 – Propionic acidemia (2026): Diagnosis, Symptoms & Billing Guide
2026 ICD-10-CM Diagnosis Code E71.121 – Propionic acidemia
What it is
Use this code for propionic acidemia, an inherited metabolic disorder where the body cannot properly break down certain amino acids and fats. It is a specific diagnosis, not a general metabolic screening result.
Clinical signs
Clinical features vary; refer to documentation. Commonly documented findings include recurrent metabolic decompensation, poor feeding, vomiting, lethargy, and developmental delay. Diagnosis is usually confirmed by metabolic testing and genetic evaluation.
When to use this code
Assign E71.121 when the provider documents propionic acidemia as the confirmed diagnosis, including during newborn, pediatric, or genetic metabolic evaluations. Use it for established disease on current admission or encounter records. If the record only notes abnormal screening or suspected disease, check documentation before coding.
Do not use for
Do not use this code for nonspecific organic acidemia, abnormal newborn screening alone, or a family history without the patient having the disorder. Check documentation if the chart only says “metabolic disorder.”
Coding tip
Code E71.121 only when the clinician clearly names propionic acidemia; otherwise, query for specificity.