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26299 Acylglycines, Quantitative, Random, Urine (AGU20)

Acylglycines, Quantitative, Random, Urine (AGU20)
Test Code: AGU20SO
Synonyms/Keywords

​2-Methylbutyryl Glycinuria
2-Methylbutyryl-CoA Dehydrogenase Deficiency
EE (Ethylmalonic Encephalopathy)
Ethylmalonic Encephalopathy (EE)
GA 1 (Glutaric Acidemia Type 1)
GA 2 (Glutaric Acidemia Type 2)
GA II (Glutaric Acidemia Type II)
GAII (Glutaric Acidemia Type 2)
GCDH (Glutaryl-CoA Dehydrogenase) Deficiency
Glutaric Acidemia (GA)
Glutaric Acidemia Type 2
Glutaric Acidemia Type I (GA I)
Glutaric Acidemia Type II (GA II)
Glutaryl-CoA Dehydrogenase (GCDH) Deficiency
Isovaleric Acidemia (IVA)
Isovaleryl-CoA Dehydrogenase (IVD)
IVA (Isovaleric Acidemia)
MADD
MCAD (Medium-Chain Acyl-CoA Dehydrogenase) Deficiency
MCKAT (Medium-Chain 3-Ketoacyl-CoA Thiolase) Deficiency
Medium-Chain 3-Ketoacyl-CoA Thiolase (MCKAT) Deficiency
Medium-Chain Acyl-CoA Dehydrogenase (MCAD) Deficiency
SBCAD (Short/Branched-Chain Acyl-CoA Dehydrogenase) Deficiency
SCAD (Short-Chain Acyl-CoA Dehydrogenase) Deficiency
Short-Chain Acyl-CoA Dehydrogenase (SCAD) Deficiency
Short/Branched-Chain Acyl-CoA Dehydrogenase (SBCAD) Deficiency
n-Acetylglycine
n-Propionylglycine
Isobutyrylglycine
Ethylmalonic acid
n-Butyrylglycine
2-Methylsuccinic acid
2-Methylbutyrylglycine
Isovalerylglycine
Glutaric acid
3-Methylcrotonylglycine
n-Tiglylglycine
3-Methylglutaconic acid
n-Hexanoylglycine
n-Octanoylglycine
3-Phenylpropionylglycine
trans-Cinnamoylglycine
Suberylglycine
Dodecanedioic acid
Tetradecanedioic acid
Hexadecanedioic acid

Useful For

Diagnosis and monitoring for patients affected with 1 of the following inborn errors of metabolism:

Fatty Acid Oxidation Disorders

-Glutaric acidemia type II

-Medium-chain 3-ketoacyl-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 (IBD) deficiency

-Isovaleryl-CoA dehydrogenase deficiency

-Multiple carboxylase deficiency

-Propionic acidemia

Highlights:

This test provides a quantitative report of abnormal levels of acylglycines in urine, identified via gas chromatography-mass spectrometry.

Specimen Requirements
Specimen TypePreferred Container/TubeAcceptable Container/TubeSpecimen VolumeSpecimen Minimum Volume
(allows for 1 repeat)
Pediatric Minimum Volume
(no repeat)
​Urine​Plastic, 10-mL urine tube​10 mL​4 mL​If insufficient collection volume, submit as much as possible in a single container; the laboratory will determine if volume is sufficient for testing.
Collection Processing Instructions

​1. Collect a random urine specimen.

2. No preservative.

Specimen Stability Information
Specimen TypeTemperatureTime
​Urine ​​Frozen (preferred)​416 days
​Refrigerated​9 days
Rejection Criteria
All specimens will be evaluated at Mayo Clinic Laboratories for test suitability. 
Performing Laboratory Information
Performing LocationDay(s) Test PerformedAnalytical TimeMethodology/Instrumentation
Mayo Clinic Laboratories​​Monday, Wednesday, Friday​5 days​Gas Chromatography-Mass Spectrometry (GC-MS) Stable Isotope Dilution Analysis
Reference Lab
Reference Range Information
Performing LocationReference Range
​Mayo Clinic Laboratories​See Mayo for reference values based on age and gender. 
Interpretation

​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; elements of differential diagnosis; recommendations for additional biochemical testing and in vitro confirmatory studies (enzyme assay, molecular analysis); name and phone number of key contacts who may provide these studies at Mayo Clinic or elsewhere; and a phone number to reach one of the laboratory directors in case the referring physician has additional questions.

Outreach CPTs
CPTModifier
(if needed)
QuantityDescriptionComments
​82542​1
Synonyms/Keywords

​2-Methylbutyryl Glycinuria
2-Methylbutyryl-CoA Dehydrogenase Deficiency
EE (Ethylmalonic Encephalopathy)
Ethylmalonic Encephalopathy (EE)
GA 1 (Glutaric Acidemia Type 1)
GA 2 (Glutaric Acidemia Type 2)
GA II (Glutaric Acidemia Type II)
GAII (Glutaric Acidemia Type 2)
GCDH (Glutaryl-CoA Dehydrogenase) Deficiency
Glutaric Acidemia (GA)
Glutaric Acidemia Type 2
Glutaric Acidemia Type I (GA I)
Glutaric Acidemia Type II (GA II)
Glutaryl-CoA Dehydrogenase (GCDH) Deficiency
Isovaleric Acidemia (IVA)
Isovaleryl-CoA Dehydrogenase (IVD)
IVA (Isovaleric Acidemia)
MADD
MCAD (Medium-Chain Acyl-CoA Dehydrogenase) Deficiency
MCKAT (Medium-Chain 3-Ketoacyl-CoA Thiolase) Deficiency
Medium-Chain 3-Ketoacyl-CoA Thiolase (MCKAT) Deficiency
Medium-Chain Acyl-CoA Dehydrogenase (MCAD) Deficiency
SBCAD (Short/Branched-Chain Acyl-CoA Dehydrogenase) Deficiency
SCAD (Short-Chain Acyl-CoA Dehydrogenase) Deficiency
Short-Chain Acyl-CoA Dehydrogenase (SCAD) Deficiency
Short/Branched-Chain Acyl-CoA Dehydrogenase (SBCAD) Deficiency
n-Acetylglycine
n-Propionylglycine
Isobutyrylglycine
Ethylmalonic acid
n-Butyrylglycine
2-Methylsuccinic acid
2-Methylbutyrylglycine
Isovalerylglycine
Glutaric acid
3-Methylcrotonylglycine
n-Tiglylglycine
3-Methylglutaconic acid
n-Hexanoylglycine
n-Octanoylglycine
3-Phenylpropionylglycine
trans-Cinnamoylglycine
Suberylglycine
Dodecanedioic acid
Tetradecanedioic acid
Hexadecanedioic acid

Ordering Applications
Ordering ApplicationDescription
If the ordering application you are looking for is not listed, contact your local laboratory for assistance.
Specimen Requirements
Specimen TypePreferred Container/TubeAcceptable Container/TubeSpecimen VolumeSpecimen Minimum Volume
(allows for 1 repeat)
Pediatric Minimum Volume
(no repeat)
​Urine​Plastic, 10-mL urine tube​10 mL​4 mL​If insufficient collection volume, submit as much as possible in a single container; the laboratory will determine if volume is sufficient for testing.
Collection Processing

​1. Collect a random urine specimen.

2. No preservative.

Specimen Stability Information
Specimen TypeTemperatureTime
​Urine ​​Frozen (preferred)​416 days
​Refrigerated​9 days
Rejection Criteria
All specimens will be evaluated at Mayo Clinic Laboratories for test suitability. 
Useful For

Diagnosis and monitoring for patients affected with 1 of the following inborn errors of metabolism:

Fatty Acid Oxidation Disorders

-Glutaric acidemia type II

-Medium-chain 3-ketoacyl-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 (IBD) deficiency

-Isovaleryl-CoA dehydrogenase deficiency

-Multiple carboxylase deficiency

-Propionic acidemia

Highlights:

This test provides a quantitative report of abnormal levels of acylglycines in urine, identified via gas chromatography-mass spectrometry.

Reference Range Information
Performing LocationReference Range
​Mayo Clinic Laboratories​See Mayo for reference values based on age and gender. 
Interpretation

​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; elements of differential diagnosis; recommendations for additional biochemical testing and in vitro confirmatory studies (enzyme assay, molecular analysis); name and phone number of key contacts who may provide these studies at Mayo Clinic or elsewhere; and a phone number to reach one of the laboratory directors in case the referring physician has additional questions.

For more information visit:
Performing Laboratory Information
Performing LocationDay(s) Test PerformedAnalytical TimeMethodology/Instrumentation
Mayo Clinic Laboratories​​Monday, Wednesday, Friday​5 days​Gas Chromatography-Mass Spectrometry (GC-MS) Stable Isotope Dilution Analysis
Reference Lab
For billing questions, see Contacts
Outreach CPTs
CPTModifier
(if needed)
QuantityDescriptionComments
​82542​1
For most current information refer to the Marshfield Laboratory online reference manual.