--- Query Results ---

Program:Genetics and Genomics
Test Name:Organic Acids, Semi-Quantitative (G&G South ACH)
Test Code:OA
Performing Site: Alberta Children's Hospital
Performing Dept:Biochemical Genetics
Availability:Test performed on a weekly basis.
TAT:14 days
Preferred Tube/Container:Random specimen: plain urine container
Alternate:24 hour urine collection container (NO ADDITIVE)
Specimen Requirements:Random Urine Collection:
Collect 5-10 mL random urine.

24 hour Urine Collection:
Urine specimen MUST be kept FROZEN during entire collection and transport process (no preservative).
Instruct patient to ensure to place 24 hour urine collection container into freezer immediately after each void throughout the 24 hour collection period.
Min. Sample Required:2.5 mL urine
Specimen Processing:Random Urine Collection:
1.  If sample is transferred to a secondary aliquot tube or container, write "random urine" on label.
2.  Freeze.

24 hour Urine Collection:
1.  Thaw 24 urine specimen.
2.  Measure total volume.
3.  Enter TVOL on requisiton.
4.  Send 10 mL aliquot of urine; write "24hr urine, Tvol #L" on the label.
5.  Freeze aliquot.
Specimen Handling:Transport frozen samples on ice. Samples must not thaw during transport.
Additional Information:Samples will be rejected if:
  • Primary container or aliquot tube received thawed
  • Sample type not written on aliquot tube label
    Requisition/Form:Submit request using Biochemical Genetics Laboratory Requisition.
    Please ensure all required information, including patient's clinical history/indication, is provided on the requisition.
    Order Restrictions:None
    Indications:This test is for the initial investigation of organic acidurias.
    This test may also be ordered to rule out the possibility of these disorders in a patient being assessed for the ketogenic diet.
    Abnormal levels found for certain organic acids present in this profile (i.e., 3-methylglutaconic or 3-methylgutaric acids, homovanillic acid, methylmalonic acid, N-acetylaspartic acid, orotic acid, vanillylmandelic acid) will be reflexively quantitated using a separate stable-isotope dilution method on the same sample, volume permitting.
    Method:Organic acids are detected following derivatization (i.e., silylation) and oximation using gas chromatography-mass spectrometry (GC-MS).
    Method Details:Metabolites reported (truncated list): lactic acid, glycolic acid, glyoxylic acid, oxalic acid, pyruvic acid, 3-hydroxybutyric acid, 2-oxovaleric acid, methylmalonic acid, 4-hydroxybutyric acid, acetoacetic acid, 2-oxo-3-methylvaleric acid, ethylmalonic acid, 2-oxoisocaproic acid, phenylacetic acid, succinic acid, methylsuccinic acid, fumaric acid, glutaric acid, 3-methylglutaric acid, succinylacetone, mandelic acid, malic acid, adipic acid, pyroglutamic acid (a.k.a., 5-oxoproline), 2-hydroxyglutaric acid, 3-hydroxy-3-methylglutaric acid, 2-oxoglutaric acid, suberic acid, 2-oxoadipic acid, orotic acid, homovanillic acid, sebacic acid, vanillylmandellic acid, 3-hydroxypropionic acid, 3-hydroxyisobutyric acid, 3-hydroxy-2-methylbutyric acid, 3-hydroxyisovaleric acid, isovalerylglycine, butyrylglycine, glyceric acid, uracil, 5-hydroxyhexanoic, thymine, propionylglycine, 3-methylglutaconic acid, tiglylglycine, oxaloacetic acid, 3-hydroxyglutaric acid, N-acetylaspartic acid, 2-hydroxyadipic acid, methylcitric acid, homogentisic acid, vanillactic acid, phenylpropionylglycine, indole-3-pyruvic acid, dodecanedoic acid, suberylglycine.
    Reference Interval:Provided on test report, based on patient age.
    Comments:Contact ACH Biochemical Genetics Laboratory (403-955-7379) for priority turn-around.
    Last Updated On:Tuesday, August 29, 2017
    Date of Last Review:Jan 2 2018 12:00AM