Acylglycines, Quantitative, Random, Urine (AGU20)

Test Code: AGU20SO

Overview
Ordering
Specimen
Performing
Clinical/Interpretive
Contacts
Coding

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

Acylglycines, Qnt, Ur (AGU20)

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 Type	Preferred Container/Tube	Acceptable Container/Tube	Specimen Volume	Specimen 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.

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 Stability Information

Specimen Type	Temperature	Time
Urine	Frozen (preferred)	416 days
Urine	Refrigerated	9 days

Rejection Criteria

All specimens will be evaluated at Mayo Clinic Laboratories for test suitability.

Performing Laboratory Information

Performing Location	Day(s) Test Performed	Report Available	Methodology/Instrumentation
Mayo Clinic Laboratories	Monday, Wednesday, Friday	5 to 14 days	Gas Chromatography-Mass Spectrometry (GC-MS) Stable Isotope Dilution Analysis

Reference Lab

Mayo Clinic Laboratories

Reference Range Information

Performing Location

Reference Range

Mayo Clinic Laboratories

n-Acetylglycine	< or = 3.50 mg/g Creatinine
n-Propionylglycine	< or = 2.25 mg/g Creatinine
Isobutyrylglycine	< or = 3.00 mg/g Creatinine
Ethylmalonic acid	< or = 25.00 mg/g Creatinine
n-Butyrylglycine	< or = 2.50 mg/g Creatinine
2-Methylsuccinic acid	< or = 9.00 mg/g Creatinine
2-Methylbutyrylglycine	< or = 2.00 mg/g Creatinine
Isovalerylglycine	< or = 8.00 mg/g Creatinine
Glutaric acid	< or = 8.00 mg/g Creatinine
3-Methylcrotonylglycine	< or = 2.25 mg/g Creatinine
n-Tiglylglycine	< or = 9.00 mg/g Creatinine
3-Methylglutaconic acid	< or = 25.00 mg/g Creatinine
n-Hexanoylglycine	< or = 2.00 mg/g Creatinine
n-Octanoylglycine	< or = 2.00 mg/g Creatinine
3-Phenylpropionylglycine	< or = 2.00 mg/g Creatinine
trans-Cinnamoylglycine	< or = 5.50 mg/g Creatinine
Suberylglycine	< or = 5.00 mg/g Creatinine
Dodecanedioic acid	< or = 0.50 mg/g Creatinine
Tetradecanedioic acid	< or = 0.50 mg/g Creatinine
Hexadecanedioic acid	< or = 0.50 mg/g Creatinine

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

CPT	Modifier (if needed)	Quantity	Description	Comments
82542		1

Synonyms/Keywords

Acylglycines, Qnt, Ur (AGU20)

Ordering Applications

Ordering Application	Description
COM	Acylglycines, Qnt. Random Urine (AGU20)
Cerner	Acylglycines, Qnt, Ur (AGU20)

If the ordering application you are looking for is not listed, contact your local laboratory for assistance.

Specimen Requirements

Specimen Type	Preferred Container/Tube	Acceptable Container/Tube	Specimen Volume	Specimen 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.

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 Stability Information

Specimen Type	Temperature	Time
Urine	Frozen (preferred)	416 days
Urine	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 Location

Reference Range

Mayo Clinic Laboratories

n-Acetylglycine	< or = 3.50 mg/g Creatinine
n-Propionylglycine	< or = 2.25 mg/g Creatinine
Isobutyrylglycine	< or = 3.00 mg/g Creatinine
Ethylmalonic acid	< or = 25.00 mg/g Creatinine
n-Butyrylglycine	< or = 2.50 mg/g Creatinine
2-Methylsuccinic acid	< or = 9.00 mg/g Creatinine
2-Methylbutyrylglycine	< or = 2.00 mg/g Creatinine
Isovalerylglycine	< or = 8.00 mg/g Creatinine
Glutaric acid	< or = 8.00 mg/g Creatinine
3-Methylcrotonylglycine	< or = 2.25 mg/g Creatinine
n-Tiglylglycine	< or = 9.00 mg/g Creatinine
3-Methylglutaconic acid	< or = 25.00 mg/g Creatinine
n-Hexanoylglycine	< or = 2.00 mg/g Creatinine
n-Octanoylglycine	< or = 2.00 mg/g Creatinine
3-Phenylpropionylglycine	< or = 2.00 mg/g Creatinine
trans-Cinnamoylglycine	< or = 5.50 mg/g Creatinine
Suberylglycine	< or = 5.00 mg/g Creatinine
Dodecanedioic acid	< or = 0.50 mg/g Creatinine
Tetradecanedioic acid	< or = 0.50 mg/g Creatinine
Hexadecanedioic acid	< or = 0.50 mg/g Creatinine

Interpretation

For more information visit:

http://labtestsonline.org

Performing Laboratory Information

Performing Location	Day(s) Test Performed	Report Available	Methodology/Instrumentation
Mayo Clinic Laboratories	Monday, Wednesday, Friday	5 to 14 days	Gas Chromatography-Mass Spectrometry (GC-MS) Stable Isotope Dilution Analysis

Reference Lab

Mayo Clinic Laboratories

For billing questions, see Contacts

Outreach CPTs

CPT	Modifier (if needed)	Quantity	Description	Comments
82542		1

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For most current information refer to the Marshfield Laboratory online reference manual.

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26299 Acylglycines, Quantitative, Random, Urine (AGU20)