ICD-10 Code E71 – Disorders of branched-chain amino-acid metabolism and fatty-acid metabolism (2026): Diagnosis, Symptoms & Billing Guide
2026 ICD-10-CM Diagnosis Code E71 – Disorders of branched-chain amino-acid metabolism and fatty-acid metabolism
What it is
E71 covers inherited disorders that affect the body’s breakdown or processing of branched-chain amino acids or fatty acids. These metabolic conditions can interfere with normal energy use and buildup of certain substances.
Clinical signs
Clinical features vary; refer to documentation. Commonly reported findings include poor feeding, vomiting, lethargy, developmental delay, or recurrent metabolic decompensation in infancy or childhood.
When to use this code
Use E71 when the record clearly identifies a disorder of branched-chain amino-acid metabolism or fatty-acid metabolism and no more specific ICD-10-CM code is documented. It is appropriate for confirmed inherited metabolic disease, not for suspected cases. If the chart names a specific disorder, code that condition instead.
Do not use for
Do not use this code for nonspecific malnutrition, transient feeding problems, or abnormal screening results without a documented diagnosis. Check documentation if the provider names a more specific metabolic disorder.
Coding tip
Code the most specific documented metabolic disorder first; use E71 only when the record supports the broader category.