ICD-10 Code E71.11 – Branched-chain organic acidurias (2026): Diagnosis, Symptoms & Billing Guide
2026 ICD-10-CM Diagnosis Code E71.11 – Branched-chain organic acidurias
What it is
E71.11 identifies branched-chain organic acidurias, a group of inherited metabolic disorders affecting breakdown of branched-chain amino acids. These conditions can lead to accumulation of organic acids in the body.
Clinical signs
Clinical features vary; refer to documentation. Common findings may include poor feeding, vomiting, lethargy, developmental delay, or metabolic decompensation in infancy or childhood.
When to use this code
Use this code when the provider documents a branched-chain organic aciduria or a specific disorder in this group and no more specific ICD-10-CM code is available. It may also fit confirmed inherited metabolic disease workups recorded as branched-chain organic aciduria. Check documentation for the exact diagnosis name.
Do not use for
Do not use this code for nonspecific amino acid abnormalities, transient metabolic disturbances, or unrelated organic acidemias. If the record names a different inherited metabolic disorder, code that condition instead.
Coding tip
Code from the provider’s exact diagnostic wording and verify whether a more specific metabolic disorder code applies.