ICD-10 Code E71.313 – Glutaric aciduria type II (2026): Diagnosis, Symptoms & Billing Guide
2026 ICD-10-CM Diagnosis Code E71.313 – Glutaric aciduria type II
What it is
Glutaric aciduria type II is an inherited metabolic disorder that impairs the body’s ability to break down certain fats and amino acids. It is also known as multiple acyl-CoA dehydrogenase deficiency.
Clinical signs
Clinical features vary; refer to documentation. Common findings include recurrent vomiting, weakness, lethargy, hypoglycemia, metabolic acidosis, and possible developmental delay or muscle hypotonia.
When to use this code
Use E71.313 when the record documents glutaric aciduria type II, multiple acyl-CoA dehydrogenase deficiency, or a confirmed inherited fatty acid oxidation disorder of this type. You may also use it when the diagnosis is established during newborn screening or metabolic workup.
Do not code from suspicion alone unless the provider documents the condition as present. If the chart only notes abnormal metabolic testing without a final diagnosis, check documentation.
Do not use for
Do not use this code for glutaric aciduria type I or other organic acidemias. Also avoid it when the record describes a nonspecific metabolic abnormality without confirmation of this disorder.
Coding tip
Code the confirmed disorder exactly as documented and verify the type, because glutaric aciduria subtypes are not interchangeable.