Sign in →

Test ID ACYLG Acylglycines, Quantitative, Urine

Useful For

Biochemical screening of asymptomatic patients affected with 1 of the following inborn errors of metabolism:

-Short chain acyl-CoA dehydrogenase (SCAD) deficiency

-Medium-chain acyl-CoA dehydrogenase (MCAD) deficiency

-Medium-chain 3-ketoacyl-CoA thiolase (MCKAT) deficiency

-Glutaric acidemia type II

-Ethylmalonic encephalopathy

-2-Methylbutyryl-CoA dehydrogenase deficiency

-Isovaleryl-CoA dehydrogenase deficiency

-Glutaryl-CoA dehydrogenase deficiency

Highlights

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

 

Diagnostic specificity of inborn errors of metabolism via urine acylglycine testing is useful only for the aforementioned inborn errors of metabolism; it is recommended that urine organic acids (OAU / Organic Acids Screen, Urine) be ordered and run simultaneously due to the limited number of metabolites included in this urine acylglycine test.

Method Name

Gas Chromatography-Mass Spectrometry (GC-MS) Stable Isotope Dilution Analysis

Reporting Name

Acylglycines, QN, U

Specimen Type

Urine

Specimen Required

Container/Tube: Plastic, 10-mL urine tube (T068)

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.

Additional Information:

1. Patient's age is required.

2. Include family history, clinical condition (asymptomatic or acute episode), diet, and drug therapy information.

Specimen Minimum Volume

4 mL

Specimen Stability Information

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

Reference Values

Control Values

Results Expressed as mg/g Creatinine

 

Range

Ethylmalonic Acid

0.5-20.2

2-Methylsuccinic Acid

0.4-13.8

Glutaric Acid

0.6-15.2

Isobutyrylglycine

0.00-11.0

n-Butyrylglycine

0.1-2.1

2-Methylbutyrylglycine

0.3-7.5

Isovalerylglycine

0.3-14.3

n-Hexanoylglycine

0.2-1.9

n-Octanoylglycine

0.1-2.1

3-Phenylpropionylglycine

0.00-1.1

Suberylglycine

0.00-11.0

trans-Cinnamoylglycine

0.2-14.7

Dodecanedioic Acid (12 DCA)

0.00-1.1

Tetradecanedioic Acid (14 DCA)

0.00-1.0

Hexadecanedioic Acid (16 DCA)

0.00-1.0

 

Day(s) and Time(s) Performed

Monday, Wednesday, Friday; 8 a.m.

Performing Laboratory

Mayo Medical Laboratories in Rochester

CPT Code Information

82542

LOINC Code Information

Test ID Test Order Name Order LOINC Value
ACYLG Acylglycines, QN, U 50334-2

 

Result ID Test Result Name Result LOINC Value
21011 Ethylmalonic Acid 13741-4
21012 2-Methylsuccinic acid 13777-8
21013 Glutaric acid 13748-9
21014 Isobutyrylglycine 38360-4
23387 n-Butyrylglycine 27892-9
21015 2-Methylbutyrylglycine 27097-5
21016 Isovalerylglycine 13766-1
23388 n-Hexanoylglycine 13753-9
21017 n-Octanoylglycine 38367-9
23389 3-Phenylpropionylglycine 13793-5
23390 Suberylglycine 13811-5
21018 trans-Cinnamoylglycine 38417-2
21019 Dodecanedioic acid 13732-3
21020 Tetradecanedioic acid 50333-4
21021 Hexadecanedioic acid 50332-6
23414 Interpretation 59462-2
23416 Reviewed By No LOINC Needed

Test Classification

This test was developed and its performance characteristics determined by Mayo Clinic in a manner consistent with CLIA requirements. This test has not been cleared or approved by the U.S. Food and Drug Administration.

Testing Algorithm

The following algorithms are available in Special Instructions:

-Newborn Screening Follow-up for Elevations of C8, C6, and C10 Acylcarnitines (also applies to any plasma or serum C8, C6, and C10 acylcarnitine elevations)

-Newborn Screening Follow-up for Isolated C4 Acylcarnitine Elevations (also applies to any plasma or serum C4 acylcarnitine elevation)

-Newborn Screening Follow-up for Isolated C5 Acylcarnitine Elevations (also applies to any plasma or serum C5 acylcarnitine elevation)