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: 30 days (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 Category

Laboratory Developed Test (LDT)

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