Test ID 2HGA 2-Hydroxyglutaric Acid Chiral Analysis, Quantitative, Random, Urine
Necessary Information
Age and sex of patient are required.
Specimen Required
Supplies: Urine Tube, 10 mL (T068)
Container/Tube: Plastic, 10-mL urine tube
Specimen Volume: 10 mL
Pediatric: If the collection volume appears insufficient, submit as much specimen as possible in a single container; the laboratory will determine if volume is sufficient for test.
Collection Instructions:
1. Collect a random urine specimen (first morning void preferred)
2. No preservative
Useful For
Determining type of 2-hydroxyglutaric aciduria by chiral analysis of urine
Highlights
This test provides differentiation of the 3 subtypes of 2-hydroxyglutaric aciduria: D-2-hydroxyglutaric aciduria, L-2-hydroxyglutaric aciduria, and combined D,L-2-hydroxyglutaric aciduria. It also provides a quantitative result of the D- and L-enantiomers of 2-hydroxyglutaric acid via gas chromatography-mass spectrometry analysis.
Method Name
Gas Chromatography-Mass Spectrometry (GC-MS)
Reporting Name
D-,L- 2-Hydroxyglutaric Acid, QN, USpecimen Type
UrineSpecimen Minimum Volume
1 mL
Specimen Stability Information
Specimen Type | Temperature | Time | Special Container |
---|---|---|---|
Urine | Frozen (preferred) | 416 days | |
Ambient | 14 days | ||
Refrigerated | 14 days |
Reference Values
Age |
D-2-hydroxyglutaric acid (mmol/mol creatinine) |
L-2-hydroxyglutaric acid |
0-11 months |
≤14.11 |
≤17.38 |
12-23 months |
≤13.76 |
≤17.03 |
24-35 months |
≤13.38 |
≤16.63 |
3 years |
≤12.96 |
≤16.18 |
4 years |
≤12.20 |
≤15.35 |
5 years |
≤11.40 |
≤14.44 |
6 years |
≤10.56 |
≤13.46 |
7 years |
≤9.71 |
≤12.43 |
8 years |
≤8.93 |
≤11.44 |
9 years |
≤8.21 |
≤10.50 |
10 years |
≤7.56 |
≤9.66 |
11 years |
≤6.99 |
≤8.94 |
12 years |
≤6.47 |
≤8.33 |
13 years |
≤6.01 |
≤7.83 |
14 years |
≤5.60 |
≤7.44 |
15 years |
≤5.23 |
≤7.14 |
16 years |
≤4.91 |
≤6.93 |
17 years |
≤4.63 |
≤6.78 |
18 years |
≤4.40 |
≤6.69 |
19 years |
≤4.21 |
≤6.63 |
20 years |
≤4.07 |
≤6.60 |
21 years |
≤3.96 |
≤6.59 |
22 years |
≤3.88 |
≤6.58 |
23 years |
≤3.81 |
≤6.56 |
24 years |
≤3.76 |
≤6.54 |
25 years |
≤3.71 |
≤6.50 |
26 years |
≤3.67 |
≤6.44 |
27 years |
≤3.63 |
≤6.37 |
28 years |
≤3.59 |
≤6.27 |
29 years |
≤3.56 |
≤6.15 |
30 years |
≤3.54 |
≤6.02 |
31 years |
≤3.52 |
≤5.87 |
32 years |
≤3.50 |
≤5.72 |
33 years |
≤3.49 |
≤5.57 |
34 years |
≤3.48 |
≤5.41 |
35 years |
≤3.46 |
≤5.26 |
36 years |
≤3.45 |
≤5.13 |
37 years |
≤3.43 |
≤5.00 |
38 years |
≤3.42 |
≤4.88 |
39 years |
≤3.40 |
≤4.78 |
40 years |
≤3.39 |
≤4.70 |
41 years |
≤3.37 |
≤4.62 |
42 years |
≤3.35 |
≤4.55 |
43 years |
≤3.33 |
≤4.50 |
44 years |
≤3.30 |
≤4.44 |
45 years |
≤3.28 |
≤4.40 |
46 years |
≤3.24 |
≤4.35 |
47 years |
≤3.21 |
≤4.31 |
48 years |
≤3.17 |
≤4.27 |
49 years |
≤3.13 |
≤4.23 |
50 years |
≤3.10 |
≤4.19 |
51 years |
≤3.07 |
≤4.16 |
52 years |
≤3.04 |
≤4.12 |
53 years |
≤3.01 |
≤4.10 |
54 years |
≤2.99 |
≤4.07 |
55 years |
≤2.97 |
≤4.04 |
56 years |
≤2.95 |
≤4.01 |
57 years |
≤2.93 |
≤3.98 |
58 years |
≤2.91 |
≤3.94 |
59 years |
≤2.89 |
≤3.91 |
60 years |
≤2.87 |
≤3.87 |
61 years |
≤2.85 |
≤3.84 |
62 years |
≤2.83 |
≤3.80 |
63 years |
≤2.79 |
≤3.78 |
64 years |
≤2.76 |
≤3.75 |
65 years |
≤2.71 |
≤3.73 |
66 years |
≤2.67 |
≤3.72 |
67 years |
≤2.61 |
≤3.71 |
68 years |
≤2.56 |
≤3.69 |
69 years |
≤2.50 |
≤3.68 |
70 years |
≤2.44 |
≤3.66 |
71 years |
≤2.38 |
≤3.64 |
72 years |
≤2.32 |
≤3.61 |
73 years |
≤2.26 |
≤3.56 |
74 years |
≤2.21 |
≤3.50 |
75 years |
≤2.16 |
≤3.43 |
76 years |
≤2.10 |
≤3.35 |
77 years |
≤2.05 |
≤3.26 |
78 years |
≤2.00 |
≤3.17 |
79 years |
≤1.94 |
≤3.08 |
80 years |
≤1.89 |
≤2.99 |
81 years |
≤1.84 |
≤2.91 |
82 years |
≤1.80 |
≤2.84 |
83 years |
≤1.75 |
≤2.78 |
84 years |
≤1.71 |
≤2.73 |
85 years |
≤1.68 |
≤2.69 |
86 years |
≤1.65 |
≤2.67 |
87 years |
≤1.64 |
≤2.65 |
88 years |
≤1.63 |
≤2.64 |
≥89 years |
≤1.62 |
≤2.64 |
Day(s) Performed
Monday
Report Available
1 to 9 daysPerforming Laboratory

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 US Food and Drug Administration.CPT Code Information
83921 x 2
LOINC Code Information
Test ID | Test Order Name | Order LOINC Value |
---|---|---|
2HGA | D-,L- 2-Hydroxyglutaric Acid, QN, U | 79297-8 |
Result ID | Test Result Name | Result LOINC Value |
---|---|---|
614619 | Interpretation | 79303-4 |
614620 | D-2-Hydroxyglutaric acid | 80100-1 |
614621 | L-2-Hydroxyglutaric acid | 80099-5 |
614622 | Reviewed By | 18771-6 |
NY State Approved
YesForms
If not ordering electronically, complete, print, and send a Biochemical Genetics Test Request (T798) with the specimen.