ICD-10 Code E71.1 – Other disorders of branched-chain amino-acid metabolism (2026): Diagnosis, Symptoms & Billing Guide
2026 ICD-10-CM Diagnosis Code E71.1 – Other disorders of branched-chain amino-acid metabolism
What it is
E71.1 identifies inherited disorders that affect breakdown of branched-chain amino acids such as leucine, isoleucine, and valine. Use it when documentation names a specific branched-chain amino-acid metabolism disorder that is not otherwise classified.
Clinical signs
Clinical features vary; refer to documentation. These disorders may present with feeding problems, vomiting, lethargy, developmental delay, or neurologic findings, but you should code only when the record supports a branched-chain amino-acid metabolic disorder.
When to use this code
Assign E71.1 when the provider documents a diagnosis such as another branched-chain amino-acid metabolism disorder, and no more specific ICD-10-CM code applies. It may also be used when the condition is confirmed by specialist evaluation or genetic/metabolic workup. Check documentation for the exact disorder name.
Do not use for
Do not use this code for general amino-acid metabolism disorders, unspecified metabolic disease, or conditions unrelated to branched-chain amino acids. If the record names a more specific disorder, code that diagnosis instead.
Coding tip
Match the provider’s exact wording and confirm whether a more specific metabolic code is available before assigning E71.1.