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Test Code LAB6754 Orotic Acid, Urine

Aliases

Argininosuccinic aciduria
Cit I
Citrullinemia I
Hereditary orotic aciduria
HHH
Hyperornithinemia, hyperammonemia, LPI
Lysinuric protein intolerance
Ornithine transcarbamylase deficiency
Uridine monophosphate synthesis (UMPS)

Purpose/Principle

Excretion of orotic acid in urine is increased in several inherited disorders of the urea cycle and in the HHH syndrome (hyperammonemia, homocitrullinuria, and hyperornithinemia). This method measures the amount of orotic acid present using stable isotope dilution methodology.

Performing Section

Biochemical Genetics

Specimen Type

Urine:
Sterile Container

Specimen Volume

Preferred: 4.0 mL
Minimum: 1.5 mL

Special Collection Notes

Random Urine, morning specimen preferred.
No preservatives

Processing Instructions

Store and ship sample frozen on dry ice.

Temperature

Frozen (dry ice)

Sample Stability

Refrigerated: 24 hours
Frozen @-20 Celsius: 30 days
Frozen @ -80 Celsius: Indefinitely

TAT

7 days

Test/Batch Schedule

Run once a week (day varies)

Test Components

Orotic Acid, Urine

Reference Range

0.0-4.6 mcg/mg UCr

CPT

82542