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
OROTICACID
Methodology

Liquid Chromatography-Tandem Mass Spectrometry (LC-MS/MS)

Performed

Thu

Reported

2-9 days

New York DOH Approval Status
This test is New York DOH approved.
Specimen Required
Patient Preparation
Collect

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.

Remarks
Stability

Ambient: Unacceptable; Refrigerated: 24 hours; Frozen: 2 weeks (avoid repeated freeze/thaw cycles)

Reference Interval
Age Orotic Acid
0-4 years 0.7-5.1 mmol/mol creatinine
5 years and older 0.2-1.5 mmol/mol creatinine

Interpretive Data



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.

Note
Hotline History
N/A
CPT Codes

83921

Components
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.
Aliases
  • 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