Orotic Acid, Urine


Sunquest Code:OROTIC  
Epic Code:LAB3647Epic Name:Orotic Acid Urine
Methodology:Liquid chromatography/Tandem mass spectrometry
Turnaround Time:Performed once per week, results are reported the next day.
Associated Links:

Urine Collection Procedure


Collection Instructions

Specimen:Urine, random or timed collection; Alternate: Filter paper, see collection instructions
Optimal Volume:Submit entire collection; analysis requires 2 mL
Container:Plastic leakproof container, sterile (Filter paper; see collection instructions)
Collection Instructions:
If using filter paper to obtain collection, obtain filter paper urine collection kit from laboratory. The kit includes one 10 x 10 cm absorbent filter paper, patient identification form, and directions for specimen collection. Collect as follows:
  • In young infants, collect from a thoroughly wet diaper not contaminated with stool. Remove top layer of diaper if disposable type. Place filter onto diaper and place diaper on flat hard surface. Place filter paper between two wet portions of diaper and press with fingertips until the filter paper becomes wet. Do this until the entire filter paper is damp (filter will look darker).
  • Ultra absorbent diapers may present a problem in collecting enough urine and are not recommended for this collection. If ultra absorbent diapers are used, place several cotton balls between the diaper and the baby's genitals. The urine in the cotton ball can then be squeezed onto the filter paper.
  • In toilet-trained children, collect 5 mL urine.
  • Place filter paper on top of plastic bag or on a clean non-absorbent surface and let filter paper dry. Label filter paper with patient name and date in pencil (do not use ink). Place dried filter paper and identification form in a plastic leakproof bag.
 


Processing and Shipping

Specimen Processing:Mix collection well before aliquot is taken. Aliquot 10 mL, 2 mL minimum. 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 Biochemical Genetics  (UMMC-East Bank)


Interpretive

Reference Range:
 
Age mmol/mol creatinine
0-4 y 0.7-5.1
5 y and older 0.2-1.5
 
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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