ICD-10 Code E71.2 – Disorder of branched-chain amino-acid metabolism, unspecified (2026): Diagnosis, Symptoms & Billing Guide
2026 ICD-10-CM Diagnosis Code E71.2 – Disorder of branched-chain amino-acid metabolism, unspecified
What it is
E71.2 identifies an unspecified disorder affecting branched-chain amino-acid metabolism, a group of inherited metabolic conditions involving leucine, isoleucine, and valine. Use it when the record confirms this metabolic disorder but does not name a more specific type.
Clinical signs
Clinical features vary; refer to documentation. Standard references describe poor feeding, vomiting, lethargy, developmental delay, or neurologic symptoms in some patients, but you should code only what is documented.
When to use this code
Use this code when the clinician documents a branched-chain amino-acid metabolism disorder without specifying the exact inherited defect or subtype. It is also appropriate when the chart confirms the diagnosis but the available record lacks enough detail for a more specific code. Check documentation if the condition is described only as an abnormal newborn screen or suspected metabolic disorder.
Do not use for
Do not use E71.2 if the documentation identifies a specific branched-chain amino-acid disorder, such as maple syrup urine disease. Do not assign it for nonspecific amino-acid abnormalities without a confirmed diagnosis.
Coding tip
Code the most specific metabolic diagnosis documented; if the subtype is missing, E71.2 is the fallback.