Orotic Acid, Urine
| Abbrev Code: | OROTIC | ||
| Order Code: | LAB3647 | Order 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. | ||
| Associated Links: | |||
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) |
Interpretive
| Reference Range: |
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| Use: |
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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