Ordering Recommendation

For proper evaluation of patients with a suspected urea cycle disorder, order Amino Acids Quantitative by LC-MS/MS, Plasma (2009389), Amino Acids Quantitative by LC-MS/MS, Urine (2009419), and Organic Acids, Urine (0098389) concurrently with orotic acid testing.


New York DOH Approval Status

This test is New York state approved.

Specimen Required

Patient Preparation

First catch urine. Also acceptable: Random urine.

Specimen Preparation

Specimen must be stored refrigerated until frozen. Transfer 2 mL urine to an ARUP standard transport tube and freeze immediately. (Min: 1 mL)

Storage/Transport Temperature

CRITICAL FROZEN. Separate specimens must be submitted when multiple tests are ordered.

Unacceptable Conditions

Urine specimens containing preservatives.


Ambient: Unacceptable; Refrigerated: 24 hours; Frozen: 30 days (avoid repeated freeze/thaw cycles)


Liquid Chromatography-Tandem Mass Spectrometry




2-9 days

Reference Interval

Age Orotic Acid
0-4 years Less than or equal to 5.1 mmol/mol creatinine
5 years and older Less than or equal to 1.5 mmol/mol creatinine

Interpretive Data

Compliance Category

Laboratory Developed Test (LDT)


Hotline History


CPT Codes



Component Test Code* Component Chart Name LOINC
0060096 Orotic Acid, Urine - Interpretation 48767-8
0092457 Orotic Acid 17869-9
3002339 Creatinine, Urine 2161-8
* Component test codes cannot be used to order tests. The information provided here is not sufficient for interface builds; for a complete test mix, please click the sidebar link to access the Interface Map.


  • Argininosuccinic aciduria
  • Cit I
  • Citrullinemia I
  • Hereditary orotic aciduria
  • HHH
  • Hyperornithinemia, hyperammonemia, LPI
  • Lysinuric protein intolerance
  • Ornithine transcarbamylase deficiency
  • UMPS
  • Uridine monophosphate synthesis
Orotic Acid, Urine