Orotic Acid and Orotidine, 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
Specimen Required
Patient Preparation
First-morning urine is preferred.  
Specimen Preparation
Urine must be refrigerated or frozen within 24 hours of collection Transport 2 mL urine. (Min: 1 mL) Freeze ASAP or within 2 hours of collection.  
Storage/Transport Temperature
CRITICAL FROZEN. Separate specimens must be submitted when multiple tests are ordered.  
Unacceptable Conditions
Urine specimens containing preservatives. Room temperature specimens.  
It is preferable that specimens be frozen immediately or as soon as possible after collection .  
Ambient: Unacceptable; Refrigerated: 24 hours; Frozen: 2 weeks (avoid repeated freeze/thaw cycles)  
Reference Interval
Effective November 14, 2011
Age Orotic Acid Orotidine
0-​4 years 0.7-​5.1 mmol/mol creatinine 0.7-​4.2 mmol/mol creatinine
5 years and older 0.2-​1.5 mmol/mol creatinine 0.2-​1.2 mmol/mol creatinine
Interpretive Data

See Compliance Statement B: www.aruplab.com/CS
Statement B: 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.
CPT Code(s)
Component Test Code*Component Chart NameLOINC
0020207Creatinine, Urine - per volume20624-3
0060096Orotic Acid, Urine - Interpretation 
0092457Orotic Acid 
* 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