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Organic Acids, Urine

Alternate test name

Lab area
Clinical Biochemistry - Metabolic Diseases
Method and equipment
Ethylacetate and ether organic acid extraction with derivatization and identification by gas chromatography-mass spectrometry (GC-MS).
Expected turn-around time
Stat: 8 hours Urgent: 2 days Routine: 3 weeks
Specimen type

Random Urine

Specimen requirements

5 mL 

Storage and transportation

Store at -20°C and transport frozen on dry ice.

Special requirements


Shipping information
The Hospital for Sick Children
Rapid Response Laboratory
555 University Avenue, Room 3642
Toronto, ON
M5G 1X8
Phone: 416-813-7200
Toll Free: 1-855-381-3212
Hours: 7 days/week, 24 hours/day
Background and clinical significance

The organic acid profile from the GC/MS can be suggestive and in many instances diagnostic for a variety of inborn errors of metabolism including Propionic acidemia, Methylmalonic acidemia, Glutaric acidemia type I, the fatty acid oxidation disorders, Tyrosinemia types I-III, PKU, Biotinidase deficiencies and many others.  

Disease condition

Various organic acidopathies

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