ICD-10 Code E71.19 – Other disorders of branched-chain amino-acid metabolism (2026): Diagnosis, Symptoms & Billing Guide
2026 ICD-10-CM Diagnosis Code E71.19 – Other disorders of branched-chain amino-acid metabolism
What it is
This code identifies inherited or acquired disorders affecting breakdown of branched-chain amino acids: leucine, isoleucine, and valine. Use it when the record describes a branched-chain amino-acid metabolic disorder but does not name a more specific diagnosis.
Clinical signs
Clinical features vary; refer to documentation. Commonly reported findings in branched-chain amino-acid disorders include poor feeding, vomiting, lethargy, developmental delay, or episodic neurologic decline.
When to use this code
Use E71.19 when the clinician documents an unspecified or other branched-chain amino-acid metabolism disorder. This is appropriate when the chart confirms the condition, but the exact subtype is not stated or does not fit a more specific code. Check documentation for neonatal, genetic, or metabolic descriptions.
Do not use for
Do not use this code for a clearly documented specific disorder such as maple syrup urine disease if a more precise code is available. Also avoid it when the note only mentions abnormal amino-acid testing without a diagnosed metabolic disorder.
Coding tip
Code the most specific documented amino-acid disorder first, and use E71.19 only when the record supports “other” or unspecified branched-chain amino-acid metabolism.