ICD-10 Code E72.22 – Arginosuccinic aciduria (2026): Diagnosis, Symptoms & Billing Guide
2026 ICD-10-CM Diagnosis Code E72.22 – Arginosuccinic aciduria
What it is
E72.22 identifies arginosuccinic aciduria, a rare inherited disorder of the urea cycle. It means the body cannot properly break down nitrogen, which can lead to toxic ammonia buildup.
Clinical signs
Clinical features vary; refer to documentation. Commonly reported findings include poor feeding, vomiting, lethargy, developmental delay, and episodes of hyperammonemia. Some patients present in infancy, while others are diagnosed later.
When to use this code
Use E72.22 when the provider documents arginosuccinic aciduria, argininosuccinate lyase deficiency, or a confirmed urea cycle disorder with this specific diagnosis. Apply it for both new and established cases when the condition is the reason for care or is clinically relevant.
Code the condition from the medical record, genetic testing, or specialist assessment. If the chart only says “metabolic disorder” or “hyperammonemia” without confirmation, check documentation before assigning this code.
Do not use for
Do not use E72.22 for nonspecific ammonia elevation, suspected metabolic disease, or other urea cycle disorders unless arginosuccinic aciduria is documented. Check documentation if the exact inherited disorder is not named.
Coding tip
Look for the exact diagnosis phrase and any specialist confirmation before coding, because “hyperammonemia” alone is not enough.