Acylglycines, Quantitative, Urine
Order Code: ACYLG
Epic Lab Code: LAB2538
Order Form: A-1a Miscellaneous Request or Epic Req
Commercial Mail-out Laboratory
01250 PFP
356-3527
Specimen(s):
Random Urine
Collection Medium:
Yellow top round bottom tube (no additive)
Minimum:
Preferred Minimum: 10 mL random urine
Absolute Minimum: 4 mL random urine
Specimen Instructions:
1. Patient's age is required.

2. Include family history, clinical condition (asymptomatic or acute episode), diet, and drug therapy information.
Turn Around Time:
5 days upon receipt at reference laboratory (not reported on Saturday or Sunday); 14 days maximum.
Reference Range:
  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 0.0-11.0 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 0.0- 1.1 Suberylglycine 0.0-11.0 trans-Cinnamoylglycine 0.2-14.7 Dodecanedioic Acid (12 DCA) 0.0- 1.1 Tetradecanedioic Acid (14 DCA) 0.0- 1.0 Hexadecanedioic Acid (16 DCA) 0.0- 1.0
Interpretive Data:
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
Comments:
Freeze urine as soon as possible; this task is performed by Specimen Control in Pathology. Avoid dilute urine when possible. Clinical information is needed for appropriate interpretation. Additional required information includes age, gender, diet (e.g., TPN therapy), drug therapy, and family history.
Methodology:
Gas Chromatography-Mass Spectrometry (GC-MS) Stable Isotope Dilution Analysis
CPT Code:
82544