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

Biohazard Infectious


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 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
(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.