Lab Test

Orotic Acid, Urine

MAYO: OROT, EPIC: LAB6120, SOFT: XORA

Instructions

Specimen Collection Criteria

Collect (preferred specimen): 24 hour urine sample with no preservative.
Also acceptable: 10 mL random urine specimen in a screw-capped container (preferred) or other sterile collection cup. (Min: 3.0 mL)

  • Keep 24 hour urine specimen iced or refrigerated during collection.
  • Include start and end dates and times for the collection period on the specimen container.
  • Critical frozen. Submit the specimen to the Laboratory immediately after the end of collection. The specimen MUST be frozen within 4 hours of collection.
Urine Preservative Options
No Preservative
6N Hydrochloric Acid
Boric Acid (10g)
Sodium Carbonate
50% Acetic Acid
REQUIRED
Unacceptable
Unacceptable
Unacceptable
Unacceptable

Rejection Criteria

  • Specimens not collected and processed as indicated. 

Performed

Wednesday.
Results available in 9-16 days.

Reference Range

By report.

Test Methodology

Colorimetric.

Interpretation

Orotic acid is an intermediate in pyrimidine biosynthesis.

Increased urinary excretion of orotic acid is observed in inborn errors of the urea cycle, with the exception of deficiency of the first enzymes in the cycle, carbamoyl-phosphate synthetase and N-acetylglutamate synthetase.

When orotic acid is measured after a protein load or allopurinol administration, excretion of orotic acid is a very sensitive indicator or ornithine carbamoyl-transferase activity.

Interpretation must be correlated with clinical and other laboratory findings.

Clinical Utility

This assay aids in the differential diagnosis of hyperammonemia and primary orotic aciduria.

CPT Code

83921.

Test Codes

MAYO: OROT, EPIC: LAB6120, SOFT: XORA

Last Updated

7/11/2019

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This directory currently reflects information only for specimens collected and/or processed at the
Farmington Hills, Grosse Pointe, Royal Oak, and Troy campuses.