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Test ID: Organic Acid Urine
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Organic Acid Urine
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Organic Acid Urine Screening
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Useful For
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Urine organic acid testing is useful for evaluating patients with possible organic acidemias, fatty acid oxidation disorders, and other conditions.
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Method name and description
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Gas Chromatography-Mass Spectrometry (GC-MS)
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Clinical information
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Organic acids occur as physiologic intermediates in a variety of metabolic pathways. Organic acidurias are a group of disorders in which one or more of these pathways are blocked, resulting in a deficiency of normal products and an abnormal accumulation of intermediate metabolites (organic acids) in the body. These excess metabolites are excreted in the urine.
Reference Mayo Clinic laboratories
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Specimen type / Specimen volume / Specimen container
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Specimen type: Random urine
Specimen Volume: 10 ml (5 ml Minimum volume).
Container/Tube: Plain urine Container, place immediately on ice after collection
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Collection instructions / Special Precautions / Timing of collection
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Collect random urine specimen , Avoid diluted urine when possible
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Relevant clinical information to be provided
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Clinical information is essential for proper interpretation.
Other required information: family history, age, gender, diet therapy and medication.
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Storage and transport instructions
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Store Frozen
Transport Temperature: Cold.
Send specimen immediately to HMC lab Central Processing (CP) areas 24/7
Lab Note: Freeze specimen immediately. Send to Metabolic lab Sunday -Thursday 7:00 AM - 02:00 PM.
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Specimen Rejection Criteria
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Inadequate patient identification (HC& Name).
Room temperature specimens.
Sample collected in wrong container (with preservative).
insufficient collection volume.
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Biological reference intervals and clinical decision values
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When no significant abnormalities are detected, the organic acid analysis is reported as “Normal Pattern”.
When abnormal results are detected, a detailed interpretation is given,including an overview of the results and of their significance, a correlation to available clinical information and recommendations for additional biochemical testing.
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Factors affecting test performance and result interpretation
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A sample collected during/immediately following an acute metabolic decompensation is likely to yield the most informative data.
Some key diagnostic compounds may be present in relatively small quantities even in asymptomatic patients – this reinforces the importance of including relevant clinical information with the Order/request.
Dietary factors such as total parenteral nutrition, medium-chain triglyceride supplementation, and ketogenic diets produce significant artifactual changes.
Medications, such as valproic acid, aspirin, and many others, can produce a wide variety of artifactual peaks.
The most common artifactual changes are due to bacterial contamination, which can be prevented by proper handling of specimens.
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Turnaround time / Days and times test performed / Specimen retention time
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Turnaround time :10 working days
Days and times test performed :Sunday to Thursday
Specimen retention time : One Month
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