ICD-10 Code E70.40 – Disorders of histidine metabolism, unspecified (2026): Diagnosis, Symptoms & Billing Guide

The ICD-10 code for Disorders of histidine metabolism, unspecified is E70.40.
2026 ICD-10-CM Diagnosis Code E70.40 – Disorders of histidine metabolism, unspecified

What it is

E70.40 identifies a disorder affecting histidine metabolism, but the specific inherited or acquired defect is not documented. Use it when records confirm a histidine-related metabolic disorder without further detail.

Clinical signs

Clinical features vary; refer to documentation. Reports may mention developmental delay, neurologic findings, or abnormal amino acid testing, but you should not assume a specific syndrome without clear provider evidence.

When to use this code

Use this code when the clinician documents a histidine metabolism disorder and does not specify the exact type. It is also appropriate when the chart notes an unspecified amino acid metabolism disorder limited to histidine. If the record names a more specific diagnosis, code that condition instead.

Do not use for

Do not use E70.40 for a confirmed, named histidine disorder when a more specific code is available. Do not assign it for general nutritional problems or abnormal labs alone; check documentation.

Coding tip

Look for the exact metabolic diagnosis in the assessment, not just test results, before selecting this unspecified code.

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