Orotic Acid, Urine
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.


Liquid Chromatography-Tandem Mass Spectrometry
2-9 days
New York DOH Approval Status
This test is New York DOH approved.
Submit With Order
ARUP Consult®
Disease Topics
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: 2 weeks (avoid repeated freeze/thaw cycles) 
Reference Interval
AgeOrotic Acid
0-4 years0.7-5.1 mmol/mol creatinine
5 years and older0.2-1.5 mmol/mol creatinine

Interpretive Data

Statement B: Compliance Statement B: For laboratory developed tests not using a RUO kit, and for FDA approved, cleared or 510(k) exempt assays with alterations. This test was developed and its performance characteristics determined by ARUP Laboratories. The U. S. Food and Drug Administration has not approved or cleared this test; however, FDA clearance or approval is not currently required for clinical use. The results are not intended to be used as the sole means for clinical diagnosis or patient management decisions.

Hotline History
View Hotline History
Component Test Code*Component Chart NameLOINC
0020207Creatinine, Urine - per volume2161-8
0060096Orotic Acid, Urine - Interpretation48767-8
0092457Orotic Acid17869-9
* 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 view this test within the Laboratory Test Directory found at www.aruplab.com
  • Argininosuccinic aciduria
  • Cit I
  • Citrullinemia I
  • Hereditary orotic aciduria
  • HHH
  • Hyperornithinemia, hyperammonemia, LPI
  • Lysinuric protein intolerance
  • Ornithine transcarbamylase deficiency
  • UMPS
  • Uridine monophosphate synthesis