ICD-10 Code E71.110 – Isovaleric acidemia (2026): Diagnosis, Symptoms & Billing Guide
2026 ICD-10-CM Diagnosis Code E71.110 – Isovaleric acidemia
What it is
E71.110 identifies isovaleric acidemia, an inherited disorder of leucine metabolism. It causes buildup of isovaleric acid and related compounds because the body cannot break them down normally.
Clinical signs
Clinical features vary; refer to documentation. Typical findings may include episodic vomiting, poor feeding, lethargy, and metabolic decompensation, especially during illness or fasting.
When to use this code
Use E71.110 when the provider documents isovaleric acidemia, isovaleric aciduria, or a confirmed inherited isovaleric acid metabolism disorder. It may also apply when the diagnosis is established by metabolic testing or genetics and appears in the assessment.
Do not assign it for nonspecific vomiting, acidosis, or newborn screening results alone unless the condition is confirmed in the record. Check documentation if the note only mentions suspected disease or family history.
Do not use for
Do not use this code for unrelated organic acidemias or for transient metabolic abnormalities without confirmed isovaleric acidemia. Check documentation if the chart uses a broader metabolic disorder term.
Coding tip
Code the confirmed inherited disorder only when the provider links the diagnosis to the encounter and avoid coding screening findings by themselves.