Other specified disorders of amino-acid metabolism

This code represents a group of rare metabolic conditions characterized by impaired processing of amino acids that are not specifically classified elsewhere. These disorders involve defects in enzymes or transport proteins leading to the accumulation of specific amino acids or their metabolites, which can result in a variety of systemic symptoms affecting multiple organ systems.

Use this code when documentation specifies an amino acid metabolism disorder that does not have a more specific ICD-10-CM code. This typically applies to conditions identified through newborn screening or diagnostic testing, such as urine organic acid analysis or plasma amino acid chromatography, confirming an unlisted amino acidopathy. Documentation should clearly state the specific amino acid disorder, if known, and indicate it falls outside of other defined categories.

• Always seek a more specific code within the E70-E72 range if available for the documented amino acid disorder.
• Ensure the medical record clearly distinguishes this from disorders of carbohydrate, lipid, or other metabolic pathways.
• Consider sequencing this code after any associated clinical manifestations or complications of the metabolic disorder.