Orotic Acid and Orotidine, Urine
0092458
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.
Mnemonic
OROTIC ACI
Methodology
Liquid Chromatography-Tandem Mass Spectrometry
Performed
Tue
Reported
2-9 days  
New York DOH Approval Status
This test is New York DOH approved.
Submit With Order
Specimen Required
Patient Preparation
  
Collect
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.  
Remarks
It is preferable that specimens be frozen immediately or as soon as possible after collection .  
Stability
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.
 
Note
 
CPT Code(s)
83921x2
Components
Component Test Code*Component Chart Name
0020207Creatinine, Urine - per volume
0060096Orotic Acid, Urine - Interpretation
0092457Orotic Acid
2005612Orotidine
* 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.
Aliases
  • Argininosuccinic aciduria
  • Cit I
  • Citrullinemia I
  • Hereditary orotic aciduria
  • HHH
  • Hyperornithinemia, hyperammonemia, LPI
  • Lysinuric protein intolerance
  • Ornithine transcarbamylase deficiency
  • UMPS
  • Uridine monophosphate synthesis