Orotic Acid, Urine

Abbrev Code:OROTIC   
Order Code:LAB3647Order Name:Orotic Acid Urine
Methodology:Liquid chromatography/Tandem mass spectrometry
CPT Codes: 83921 x1
Turnaround Time:Performed once per week, results are reported the next day.
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Urine Collection Procedure

Collection Instructions

Specimen:Urine, random or timed collection
Optimal Volume:Submit entire collection; analysis requires 2 mL
Container:Plastic leakproof container, sterile

Processing and Shipping

Specimen Processing:Mix collection well before aliquot is taken. Aliquot urine for UCRR and aliquot 10 mL, 2 mL minimum to send to Biochemical Genetics. Room temperature or refrigerated samples must arrive at Biochemical Genetics within 2 hours of collection (preferred). Alternately, store specimen in the freezer and ship on ice.
Shipping Instructions:Ship on dry ice.
Test Performed at or Referral Lab UM Special Drug/Biochemical Genetics (Y)  (UMMC East Bank)


Reference Range:
Age mmol/mol creatinine
0-4 y 0.7-5.1
5 y and older 0.2-1.5
Urinary orotic acid excretion may be useful in screening for urea cycle disorders and urinary orotic acid excretion combined with serum and urine amino acid quantitation can be helpful in localizing defects in patients with urea cycle disorders. This test is positive in hereditary orotic aciduria, ornithine carbamyl transferase deficiency, and argininemia. It is variably positive in other urea cycle defects except carbamyl phosphate synthetase deficiency.  

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