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Denver Genetic Laboratories

Orotic Acid, Quantitative, Urine

Clinical Test Information - CHCO Biochemical Genetics Laboratory


CPT Code:



Monday - Friday, 0800 - 1700.

Turnaround Time:

2 - 3 days.

Minimum Volume:

1.0 mL (at least 2.0 mL preferred).

Specimen Handling:

Random urine, morning specimen preferred.  Freeze without preservative.

Interpretive Reference Range:

Normal < 4.7 mg/mg creatinine.


Useful, together with serum and urine amino acids, in localizing urea cycle defects. Increased in ornithine transcarbamylase (OTC) deficiency, citrullinemia, argininosuccinic acidemia,
HHH (hyperornithinemia-hyperammonemia-homocitrullinuria) syndrome, and hereditary orotic aciduria.


Stable isotope dilution gas chromatography-mass spectrometry (GC/MS).