Alberta Precision Laboratories

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Test Name/Synonym:

Organic Acids, Semi-Quantitative (Synonyms: Homogentisic, Succinylacetone, Pyroglutamic, Oxoproline, Glutaric)

Clinical Indications:

This test is for the investigation of organic acidurias and certain fatty acids oxidation disorders.

This test may also be ordered to rule out the possibility of these disorders in a patient being assessed for the ketogenic diet.

Test Includes:

Metabolites reported (truncated list):

2-hydroxyadipic acid	2-hydroxyglutaric acid	2-oxoadipic acid
2-oxoglutaric acid	2-oxoisocaproic acid	2-oxovaleric acid
2-oxo-3-methylvaleric acid	3-hydroxy-2-methylbutyric acid	3-hydroxy-3-methylglutaric acid
3-hydroxybutyric acid	3-hydroxyglutaric acid	3-hydroxyisobutyric acid
3-hydroxyisovaleric acid	3-hydroxypropionic acid	3-methylglutaconic acid
3-methylglutaric acid	4-hydroxybutyric acid	5-hydroxyhexanoic
acetoacetic acid	adipic acid	butyrylglycine
dodecanedioic acid	ethylmalonic acid	fumaric acid
glutaric acid	glyceric acid	glycolic acid
glyoxylic acid	homogentisic acid	homovanillic acid
indole-3-pyruvic acid	isovalerylglycine	lactic acid
malic acid	mandelic acid	methylcitric acid
methylmalonic acid	methylsuccinic acid	N-acetylaspartic acid
orotic acid	oxalic acid	oxaloacetic acid
phenylacetic acid	phenylpropionylglycine	propionylglycine
pyroglutamic acid (a.k.a., 5-oxoproline)	pyruvic acid	sebacic acid
suberic acid	suberylglycine	succinic acid
succinylacetone	thymine	tiglylglycine
uracil	vanillic acid	vanillylmandelic acid

LABID (Connect Care):

LAB1059A

Test Code:

CALGARY ZONE	BGLNON; IN BGL TEST NAME FIELD ENTER SEMI-QUANT OA
CENTRAL ZONE	ORGACUR
SOUTH ZONE	ORGACURT
OTHER ZONES	NOT SPECIFIED

Specimen Type:

Urine

Primary Container:

Sterile container

Alternate Containers:

Non sterile container

Minimum Collection Volume:

2.5 mL

Specimen Collection Requirements:

First morning void is preferred.
Collect a 5.0 to 10.0 mL random urine specimen.

Test Resources:

Submit request using the applicable requisition or Connect Care online order entry:

Biochemical Genetics Laboratory Requisition

Please ensure all required information, including patient's clinical history/indication, is provided on the requisition.

Stability and Storage:

Frozen

Processing:

This test requires creatinine determination for result reporting. For sites using EPIC, a separate aliquot should be removed at the time of initial specimen receipt and sent to the EPIC-designated lab for creatinine testing.

One aliquot with primary container, or two separate aliquots, can be submitted. Write “urine” on all secondary aliquot tubes. Freeze container and/or tubes.

Transportation:

Transport frozen specimens on dry ice.
Samples must not thaw during transport.

Method:

Gas chromatography-mass spectrometry (GC-MS)

Method and Interpretation of Results:

Organic acids are detected following derivatization (i.e., silylation) and oximation using gas chromatography-mass spectrometry (GC-MS).

An overall interpretation of the organic acid profile analyzed semi-quantitatively by this method will be provided by a biochemical geneticist.

Abnormal levels found for certain organic acids present in this profile (i.e., 3-methylglutaconic or 3-methylgutaric acids, methylmalonic acid, N-acetylaspartic acid, orotic acid) will be reflexively quantitated using a separate stable-isotope dilution method on the same sample, volume permitting.

Routine Turn Around Time:

14 days

Testing Schedule:

Weekly

Reference Interval:

Provided on test report, based on patient age.

Testing Area:

Biochemical Genetics

Performing Site:

Alberta Children’s Hospital

Additional Test Information:

For priority turn-around contact Biochemical Genetics South Laboratory.

Contact Comments:

Biochemical Genetics 403-955-7379

Last Updated On:

Monday, September 19, 2022

Date of Last Review:

Aug 1 2022 12:00AM