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, U

Specimen Type

Urine

Specimen Minimum Volume

1 mL

Specimen Stability Information

Specimen Type	Temperature	Time
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 (mmol/mol creatinine)
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 days

Performing Laboratory

Mayo Clinic Laboratories in Rochester

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

Yes

Forms

If not ordering electronically, complete, print, and send an Inborn Errors of Metabolism Test Request (T798) with the specimen.

Mayo Clinic Laboratories Rochester

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Test ID 2HGA 2-Hydroxyglutaric Acid Chiral Analysis, Quantitative, Random, Urine