Test of Urine Organic Acids
| Test Code | UO |
|---|---|
| Test Summary | Detecting urine organic acids by GC-MS/MS. |
| Turn Around Time | 5 days |
| Acceptable Sample Types | Urine Filter Paper |
| NY Approved | No |
| Accreditations | EQA |
*TAT starts after the sample and all required sample information is received at the processing laboratory.
**The CPT codes listed are in accordance with Current Procedural Terminology, a publication of the American Medical Association, and are provided for informational purposes only. CPT coding is the sole responsibility of the billing party.
This testing service has not been cleared or approved by the U.S. Food and Drug Administration. Testing services may not be licensed in accordance with the laws in all countries. The availability of specific test offerings is dependent upon laboratory location.
**The CPT codes listed are in accordance with Current Procedural Terminology, a publication of the American Medical Association, and are provided for informational purposes only. CPT coding is the sole responsibility of the billing party.
This testing service has not been cleared or approved by the U.S. Food and Drug Administration. Testing services may not be licensed in accordance with the laws in all countries. The availability of specific test offerings is dependent upon laboratory location.
This test is not available in your region Japan, please select your nearest laboratory to request more information.
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Test Code:
UO
Test Description
The analysis of urine organic acids by gas chromatography-mass spectrometry (GC-MS) can detect >130 analytes, and assists in the diagnosis of many inherited metabolic diseases. Urine organic acid testing is useful in the diagnosis and monitoring of patients with inborn errors of organic acid metabolism, inborn errors of amino acid metabolism, urea cycle defects, and defects of the mitochondrial respiratory chain. Organic acid analysis may fail to detect certain disorders that are characterized by minimal or intermittent metabolite excretion.
Indications for Testing
- Neonatal genetic metabolic disease screening results are abnormal.
- Children and adults with suspected inherited metabolic diseases.
Condition Description
Organic acidemia is a term used to classify a group of metabolic disorders that disrupt normal amino acid metabolism, particularly branched-chain amino acids, causing a buildup of acids that are usually not present. The four main types of organic acidemia are: methylmalonic acidemia, propionic acidemia, isovaleric acidemia, and maple syrup urine disease. Most of the organic acidemias result from defective autosomal genes for various enzymes important to amino acid metabolism. Organic acidemias are usually diagnosed in infancy, characterized by urinary excretion of abnormal amounts or types of organic acids. These disorders vary in their prognosis, from manageable to fatal, and usually affect more than one organ system, especially the central nervous system. Neurological damage and developmental delay are common factors in diagnosis, with associated symptoms ranging from poor feeding to slow growth, lethargy, vomiting, dehydration, malnutrition, hypoglycemia, hypotonia, metabolic acidosis, ketoacidosis, hyperammonemia, and if left untreated, death.
Test Methods and Limitations
Gas chromatography–mass spectrometry (GC–MS) is an analytical method that combines the features of gas-chromatography and mass spectrometry to identify different substances within a test sample.