ICD-10 Code E72.2 – Disorders of urea cycle metabolism (2026): Diagnosis, Symptoms & Billing Guide

The ICD-10 code for Disorders of urea cycle metabolism is E72.2.
2026 ICD-10-CM Diagnosis Code E72.2 – Disorders of urea cycle metabolism

What it is

E72.2 identifies inherited disorders that impair the urea cycle, the body’s pathway for removing nitrogen. When this pathway fails, ammonia can build up in the blood and cause metabolic illness.

Clinical signs

Clinical features vary; refer to documentation. Commonly reported findings include poor feeding, vomiting, lethargy, confusion, and other signs of hyperammonemia, especially after illness or increased protein intake.

When to use this code

Use E72.2 when the provider documents a urea cycle disorder, such as a specific inherited defect in ammonia detoxification. It also fits confirmed metabolic disease with documented urea cycle involvement. If the record only mentions elevated ammonia without a diagnosed disorder, check documentation before coding.

Do not use for

Do not use this code for nonspecific hyperammonemia, liver disease, or other metabolic disorders without urea cycle involvement. If the diagnosis is uncertain or only suspected, check documentation.

Coding tip

Code the confirmed disorder as documented and capture any named subtype when available.

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