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Test #


Test CPT Codes

82570, 83921

Orotic Acid, Urine

Test Type

Organic Acid Analysis (Biochemical & Molecular Genetics)

Test Disease / Components

Urea cycle disorders (including but not limited to ornithine transcarbamylase (OTC) deficiency, citrullinemia, and argininosuccinic aciduria), lysinuric protein intolerance, hyperornithinemia, hyperammonemia, homocitrullinuria [HHH] syndrome, and hereditary orotic aciduria

Test Technology

Gas Chromatogram/Mass Spectrometry (GC/MS)

Clinical Utility

Diagnostic, Monitoring

Test Specimen


  • Preferred: Random urine, no preservative in a plastic collection container

  • Acceptable: Aliquot from a 24 hour urine collection, no preservative

  • 5.0 mL urine minimum volume

Storage, Stability & Shipping


  • Refrigerate following collection


  • Up to 24 hours at room temperature

  • 5 days refrigerated

  • 1 year frozen


  • Local Clients: Transport in a cooler with ice packs within 24 hours of collection

  • Out-of-area Clients: Specimens should be shipped frozen on dry ice

Turnaround Time (TAT)

72 Hours

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