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

Canada

M5G 1X8

Phone: 416-813-7200

Toll Free: 1-855-381-3212

Hours: 7 days/week, 24 hours/day

Requisition

Organic Acids, Urine - requisition

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