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HelpTest Code LAB3594 Acylglycines, Quantitative, Random, Urine
Additional Testing Requirements
Diagnostic specificity of inborn errors of metabolism via urine acylglycine testing is available only for selected inborn errors of metabolism; it is recommended that urine organic acids (OAU / Organic Acids Screen, Random, Urine) be ordered and assessed simultaneously due to the limited number of metabolites included in this urine acylglycine test.
Necessary Information
1. Patient's age and sex are required.
2. Include family history, clinical condition (asymptomatic or acute episode), diet, and drug therapy information
Specimen Required
Supplies: Urine Tubes, 10 mL (T068)
Container/Tube: Plastic, 10-mL urine tube
Specimen Volume: 10 mL
Pediatric: If insufficient collection volume, submit as much specimen as possible in a single container; the laboratory will determine if volume is sufficient for testing.
Collection Instructions:
1. Collect a random urine specimen.
2. No preservative.
Forms
1. Biochemical Genetics Patient Information (T602)
2. If not ordering electronically, complete, print, and send a Biochemical Genetics Test Request (T798) with the specimen.
Useful For
Diagnosis and monitoring for patients affected with one of the following inborn errors of metabolism:
Fatty Acid Oxidation Disorders:
-Glutaric acidemia type II
-Medium-chain 3-ketoacyl-coenzyme A (CoA) thiolase (MCKAT) deficiency
-Medium-chain acyl-CoA dehydrogenase (MCAD) deficiency
-Short chain acyl-CoA dehydrogenase (SCAD) deficiency
Organic Acidurias:
-2-Methyl-3-hydroxybutyryl-CoA dehydrogenase (2M3HBD) deficiency
-2-Methylbutyryl-CoA dehydrogenase deficiency
-3-Methylcrotonyl-CoA carboxylase deficiency
-3-Methylglutaconyl-CoA-hydratase deficiency
-Aminoacylase 1 deficiency
-Beta-ketothiolase deficiency
-Ethylmalonic encephalopathy
-Glutaryl-CoA dehydrogenase deficiency
-Isobutyryl-CoA dehydrogenase deficiency
-Isovaleryl-CoA dehydrogenase deficiency
-Multiple carboxylase deficiency
-Propionic acidemia
Special Instructions
Method Name
Gas Chromatography Mass Spectrometry (GC-MS)
Reporting Name
Acylglycines, QN, USCH Clinical System Name: Acylglycines Quantitation, Urine
Specimen Type
UrineSpecimen Minimum Volume
4 mL
Specimen Stability Information
| Specimen Type | Temperature | Time |
|---|---|---|
| Urine | Frozen (preferred) | 416 days |
| Refrigerated | 9 days |
Reject Due To
All specimens will be evaluated at Mayo Clinic Laboratories for test suitability.Reference Values
n-Acetylglycine:≤3.50 mg/g Creatinine
n-Propionylglycine:≤2.25 mg/g Creatinine
Isobutyrylglycine:≤3.00 mg/g Creatinine
Ethylmalonic acid:≤25.00 mg/g Creatinine
n-Butyrylglycine:≤2.50 mg/g Creatinine
2-Methylsuccinic acid:≤9.00 mg/g Creatinine
2-Methylbutyrylglycine:≤2.00 mg/g Creatinine
Isovalerylglycine:≤8.00 mg/g Creatinine
Glutaric acid:≤8.00 mg/g Creatinine
3-Methylcrotonylglycine:≤2.25 mg/g Creatinine
n-Tiglylglycine:≤9.00 mg/g Creatinine
3-Methylglutaconic acid:≤25.00 mg/g Creatinine
n-Hexanoylglycine:≤2.00 mg/g Creatinine
n-Octanoylglycine:≤2.00 mg/g Creatinine
3-Phenylpropionylglycine:≤2.00 mg/g Creatinine
trans-Cinnamoylglycine:≤5.50 mg/g Creatinine
Suberylglycine:≤5.00 mg/g Creatinine
Dodecanedioic acid:≤0.50 mg/g Creatinine
Tetradecanedioic acid:≤0.50 mg/g Creatinine
Hexadecanedioic acid:≤0.50 mg/g Creatinine
Method Description
Urine volumes equivalent to 0.25 to 0.50 mg of creatinine are spiked with the mixture of labeled internal standards, allowed to equilibrate, acidified, and then extracted. After evaporation, the dry residue is derivatized to butyl esters. Specimens are analyzed by capillary gas chromatography mass spectrometry selected ion monitoring using ammonia chemical ionization and a stable isotope dilution method.(Unpublished Mayo method)
Performing Laboratory
Mayo Clinic Laboratories in Rochester
Test Classification
This test was developed and its performance characteristics determined by Mayo Clinic in a manner consistent with CLIA requirements. It has not been cleared or approved by the US Food and Drug Administration.CPT Code Information
82542
LOINC Code Information
| Test ID | Test Order Name | Order LOINC Value |
|---|---|---|
| AGU20 | Acylglycines, QN, U | 50334-2 |
| Result ID | Test Result Name | Result LOINC Value |
|---|---|---|
| 608931 | Interpretation | 53718-3 |
| 608910 | n-Acetylglycine | 99059-8 |
| 608911 | n-Propionylglycine | 13800-8 |
| 608912 | Isobutyrylglycine | 38360-4 |
| 608913 | Ethylmalonic acid | 13741-4 |
| 608914 | n-Butyrylglycine | 27892-9 |
| 608915 | 2-Methylsuccinic acid | 13777-8 |
| 608916 | 2-Methylbutyrylglycine | 27097-5 |
| 608917 | Isovalerylglycine | 13766-1 |
| 608918 | Glutaric acid | 13748-9 |
| 608919 | 3-Methylcrotonylglycine | 13691-1 |
| 608920 | n-Tiglylglycine | 13816-4 |
| 608921 | 3-Methylglutaconic acid | 13692-9 |
| 608922 | n-Hexanoylglycine | 13753-9 |
| 608923 | n-Octanoylglycine | 38367-9 |
| 608924 | 3-Phenylpropionylglycine | 13793-5 |
| 608925 | trans-Cinnamoylglycine | 38417-2 |
| 608926 | Suberylglycine | 13811-5 |
| 608927 | Dodecanedioic acid | 13732-3 |
| 608928 | Tetradecanedioic acid | 50333-4 |
| 608929 | Hexadecanedioic acid | 50332-6 |
| 608930 | Reviewed By | 18771-6 |
Day(s) Performed
Monday, Thursday
Report Available
3 to 6 daysTesting Algorithm
For more information see Newborn Screen Follow-up for Elevated C5-OH.