Orotic Acid, Urine

Last Modified: 10/6/2017 4:08:35 PM


Specimen Requirements:

10.0 mL Urine Collection in a Non-Sterile Yellow Capped Container
Collection Instructions: For this test a patient's age is required with normal required information of the order.

Urine could be a random or timed urine specimen
Minimum Volume: 3.0 mL Urine
Transport & Storage: Temperature/Stability: 30 days Frozen
Reference Range:

<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
> or =11 years: 0.4-1.2 mmol/mol creatinine

The value for the orotic acid concentration is reported. The interpretation of the result must be correlated with clinical and other laboratory findings
Test Comments: Pregnant women will normally excrete up to twice the upper limit of the adult reference range.
Methodology: Colorimetric
Clinical Significance:

Evaluation of the differential diagnosis of hyperammonemia and hereditary orotic aciduria

When orotic acid is measured after a protein load or administration of allopurinol, excretion of orotic acid is a very sensitive indicator of ornithine transcarbamylase (OTC) activity. An allopurinol challenge may be helpful in determining whether a female patient may be a carrier of an OTC mutation if molecular genetic testing was not informative.
Custom Panel: No

PRODUCTION SCHEDULE

Turn Around Time: 4 to 8 days
Days Performed: Tuesday, Thursday
Sites Performed: Mayo Laboratory
PHL Test Code: MSOT
EPIC Test Code:
Send Out Test Code: OROT - 8905
Alternate Test Names: Argininosuccinic Aciduria; ASA (Aspirin); Cit I; Citrullinemia I; Hereditary Orotic Aciduria; HHH; Hyperornithinemia, Hyperammonemia, Homocitrullinur; LPI; Lysinuric Protein Intolerance; Ornithine Transcarbamylase Deficiency; OTC (Ornithine Transcarbamylase); UMPS; Urea Cycle Disorder (UCD); Uridine Monophosphate Synthase Deficiency
CPT Coding: 83921

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