2-Hydroxyglutaric Acid Chiral Analysis, Quantitative, Random, Urine

Necessary Information

1. Age and sex of patient are required.

2. Biochemical Genetics Patient Information (T602) is recommended, but not required, to be filled out and sent with the specimen to aid in the interpretation of test results.

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

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

Reject Due To

All specimens will be evaluated at Mayo Clinic Laboratories for test suitability.

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

Method Description

Urine volumes are spiked with a mixture of internal standards and evaporated. The dry residue is derivatized to form the diastereomeric molecules, then acidified and extracted. After evaporation, the dry residue is derivatized esters at the carboxylic acid moiety. Specimens are then analyzed by capillary gas chromatography mass spectrometry selected ion monitoring using chemical ionization, with chromatographic separation of the L,L- and D,L-diastereoisomers of derivatized 2-hydroxyglutaric acid (2-HGA), corresponding to L-2-HGA and D-2-HGA, respectively.(Unpublished Mayo method)

Day(s) Performed

Thursday

Performing Laboratory

Mayo Clinic Laboratories in Rochester

CPT Code Information

83921 x 2

Forms

1. Biochemical Genetics Patient Information (T602)

2. If not ordering electronically, complete, print, and send a Biochemical Genetics Test Request (T798) with the specimen.

Special Instructions

Biochemical Genetics Patient Information

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Order Code 2HGA 2-Hydroxyglutaric Acid Chiral Analysis, Quantitative, Random, Urine