Ordering Recommendation

Diagnose and monitor for fatty acid oxidation disorders and organic acidemias. Use in conjunction with urine organic acids and plasma acylcarnitines testing.

New York DOH Approval Status

This test is New York state approved.

Specimen Required

Patient Preparation

Random urine.

Specimen Preparation

Transfer 6 mL urine to ARUP Standard Transport Tubes and freeze immediately. (Min: 3 mL) Avoid dilute urine when possible.

Storage/Transport Temperature


Unacceptable Conditions

Clinical information is needed for appropriate interpretation. Additional required information includes age, gender, diet (e.g., TPN therapy), drug therapy, and family history. Biochemical Genetics Patient History Form is available on the ARUP Web site at https://www.aruplab.com/patienthistory or by contacting ARUP Client Services.


Ambient: Unacceptable; Refrigerated: 24 hours; Frozen: 30 days


Liquid Chromatography-Tandem Mass Spectrometry




2-13 days

Reference Interval

Reports include age appropriate reference interval.

Test Number
Reference Interval
  Propionylglycine By report
  Isobutyrylglycine By report
  Butyrylglycine By report
  2-Methylbutyrylglycine By report
  Isovalerylglycine By report
  3-Methylcrotonylglycine By report
  Tiglylglycine By report
  Hexanoylglycine By report
  Phenylpropionylglycine By report
  Suberylglycine By report

Interpretive Data

This test was developed and its performance characteristics determined by ARUP Laboratories. It has not been cleared or approved by the US Food and Drug Administration. This test was performed in a CLIA certified laboratory and is intended for clinical purposes.

Compliance Category

Laboratory Developed Test (LDT)


Hotline History


CPT Codes



Component Test Code* Component Chart Name LOINC
0081171 Acylglycines, Urine Interpretation 53718-3
0081172 Propionylglycine 24442-6
0081173 Isobutyrylglycine 24439-2
0081174 Butyrylglycine 24437-6
0081175 2-Methylbutyrylglycine 24435-0
0081176 Isovalerylglycine 24440-0
0081177 3-Methylcrotonylglycine 24436-8
0081178 Tiglylglycine 24444-2
0081179 Hexanoylglycine 24438-4
0081180 Phenylpropionylglycine 24441-8
0081181 Suberylglycine 24443-4
3002335 Creatinine, Urine 2161-8
* Component test codes cannot be used to order tests. The information provided here is not sufficient for interface builds; for a complete test mix, please click the sidebar link to access the Interface Map.


  • quantiative analysis of urinary acylglycines
  • urinary acylcarnitines
Acylglycines, Quantitative, Urine