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Test Code OROT Orotic Acid, Random, Urine

Reporting Name

Orotic Acid, U

Useful For

Evaluation of the differential diagnosis of hyperammonemia and hereditary orotic aciduria

 

Sensitive indicator of ornithine transcarbamylase (OTC) activity after administration of allopurinol or a protein load to identify OTC carriers

Performing Laboratory

Mayo Clinic Laboratories in Rochester

Specimen Type

Urine


Necessary Information


1. Patient's age is required.

2. Provide a reason for testing.



Specimen Required


Supplies: Urine Tubes, 10 mL (T068)

Container/Tube: Plastic, 10-mL urine tube

Specimen Volume: 10 mL

Collection Instructions:

1. Collect a random or timed urine specimen.

2. No preservative needed.


Specimen Minimum Volume

3 mL

Specimen Stability Information

Specimen Type Temperature Time Special Container
Urine Frozen 30 days

Reference Values

<2 weeks: 1.4-5.3 mmol/mol creatinine

2 weeks-1 year: 1.0-3.2 mmol/mol creatinine

2-10 years: 0.5-3.3 mmol/mol creatinine

≥11 years: 0.4-1.2 mmol/mol creatinine

Day(s) Performed

Tuesday, Thursday

Test Classification

This test was developed and its performance characteristics determined by Mayo Clinic in a manner consistent with CLIA requirements. It has not been cleared or approved by the US Food and Drug Administration.

CPT Code Information

83921

LOINC Code Information

Test ID Test Order Name Order LOINC Value
OROT Orotic Acid, U 17869-9

 

Result ID Test Result Name Result LOINC Value
8905 Orotic Acid, U 17869-9

Interpretation

The value for the orotic acid concentration is reported. The interpretation of the result must be correlated with clinical and other laboratory findings.

Clinical Reference

1. Singh RH, Rhead WJ, Smith W, et al. Nutritional management of urea cycle disorders. Crit Care Clin. 2005;21(4 Suppl):S27-35

2. Lee B, Singh RH, Rhead WJ, et al. Considerations in the difficult-to-manage urea cycle disorder patient. Crit Care Clin. 2005;21(4 Suppl):S19-25

3. Brusilow SW, Horwich AL. Urea cycle enzymes. In: Valle D, Antonarakis S, Ballabio A, Beaudet AL, Mitchell GA, eds. The Online Metabolic and Molecular Bases of Inherited Disease. McGraw-Hill; 2019. Accessed January 14, 2024. Available at http://ommbid.mhmedical.com/content.aspx?bookid=2709&sectionid=225084071

4. Webster DR, Becroft DO, van Gennip AH, Van Kuilenburg AP. Hereditary orotic aciduria and other disorders of pyrimidine metabolism. In: Valle D, Antonarakis S, Ballabio A, Beaudet AL, Mitchell GA, eds. The Online Metabolic and Molecular Bases of Inherited Disease., McGraw-Hill; 2019. Accessed January 14, 2024. Available at http://ommbid.mhmedical.com/content.aspx?bookid=2709&sectionid=225090376

5. Ah Mew N, Simpson KL, Gropman AL, Lanpher BC, Chapman KA, Summar, ML. Urea cycle disorders overview. In: Adam MP, Ardinger HH, Pagon RA, et al, eds. GeneReviews [Internet]. University of Washington, Seattle; 2003. Updated June 22, 2017. Accessed January 14, 2024. Available at www.ncbi.nlm.nih.gov/books/NBK1217

Report Available

4 to 8 days

Method Name

Colorimetric