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HelpTest Code AGU20 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.
CMH COLLECTION: 4mL urine in sterile cup
Specimen Minimum Volume
4 mL
Specimen Stability Information
| Specimen Type | Temperature | Time | Special Container |
|---|---|---|---|
| Urine | Frozen (preferred) | 416 days | |
| Refrigerated | 9 days |
Testing Algorithm
For more information see Newborn Screen Follow-up for Elevated C5-OH.
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
Reject Due To
All specimens will be evaluated at Mayo Clinic Laboratories for test suitability.Day(s) Performed
Monday, Thursday
Report Available
3 to 6 daysReporting Name
Acylglycines, QN, UReference 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 Name
Gas Chromatography Mass Spectrometry (GC-MS)
Performing Laboratory
Mayo Clinic Laboratories in Rochester
CPT Code Information
82542