Identification ofN-acetyl-α-aminoadipic acid in the urine of a patient with α-aminoadipic and α-ketoadipic aciduria

A 3.5-year-old Japanese boy with a mild speech disturbance excreted a large amount of α-aminoadipic acid into the urine. The amino acid analysis using an amino acid analyser confirmed the presence of α-aminoadipic acid in both urine and plasma.