Ordering Recommendation

Aid in the diagnosis of selected neurotransmitter disorders (eg, glycine encephalopathy, brain serine deficiency).

New York DOH Approval Status

This test is New York state approved.

Specimen Required

Patient Preparation
Collect

CSF.

Specimen Preparation

Centrifuge CSF to separate and remove cellular material. Freeze supernatant. Transport 0.5 mL CSF. (Min: 0.3 mL)

Storage/Transport Temperature

CRITICAL FROZEN. Separate specimens must be submitted when multiple tests are ordered.

Unacceptable Conditions
Remarks

Clinical information is needed for appropriate interpretation. Additional required information includes age, gender, diet (e.g., TPN therapy), drug therapy, and family history. Biochemical Genetics Patient History Form is available on the ARUP Web site at https://www.aruplab.com/patienthistory or by contacting ARUP Client Services.

Stability

Ambient: Unacceptable; Refrigerated: 24 hours; Frozen: 1 month

Methodology

Liquid Chromatography-Tandem Mass Spectrometry

Performed

Sun-Sat

Reported

2-5 days

Reference Interval

Effective November 18, 2019

Components Reference Interval Components Reference Interval
Alpha-Aminoadipic acid Less than or equal to 2.0 µmol/L Histidine 7.0 - 24.0 µmol/L
Alpha-Amino-n-butyric acid Less than or equal to 8.0 µmol/L Homocitrulline Less than or equal to 5.0 µmol/L
Alanine 16.0 - 46.0 µmol/L Homocystine Less than or equal to 2.0 µmol/L
Alloisoleucine Less than or equal to 2.0 µmol/L Hydroxylysine Less than or equal to 5.0 µmol/L
Anserine Less than or equal to 7.0 µmol/L Hydroxyproline Less than or equal to 4.0 µmol/L
Arginine 8.0 - 31.0 µmol/L Isoleucine Less than or equal to 12.0 µmol/L
Argininosuccinic acid Less than or equal to 2.0 µmol/L Leucine 5.0 - 22.0 µmol/L
Asparagine 4.0 - 13.0 µmol/L Lysine 10.0 - 36.0 µmol/L
Aspartic acid Less than or equal to 5.0 µmol/L Methionine 2.0 - 7.0 µmol/L
Beta-Alanine Less than or equal to 25.0 µmol/L Ornithine Less than or equal to 10.0 µmol/L
Beta-Aminoisobutyric acid Less than or equal to 5.0 µmol/L Phenylalanine 6.0 - 20.0 µmol/L
Citrulline Less than or equal to 5.0 µmol/L Proline Less than or equal to 5.0 µmol/L
Cystathionine Less than or equal to 5.0 µmol/L Sarcosine Less than or equal to 5.0 µmol/L
Cystine Less than or equal to 5.0 µmol/L Serine 18.0 - 66.0 µmol/L
Ethanolamine Less than or equal to 40.0 µmol/L Taurine Less than or equal to 13.0 µmol/L
Gamma-Amino-n-butyric acid Less than or equal to 5.0 µmol/L Threonine 14.0 - 59.0 µmol/L
Glutamic acid Less than or equal to 5.0 µmol/L Tryptophan Less than or equal to 5.0 µmol/L
Glutamine 330.0 - 630.0 µmol/L Tyrosine 5.0 - 23.0 µmol/L
Glycine 5.0 - 20.0 µmol/L Valine 8.0 - 30.0 µmol/L

Interpretive Data

This test was developed and its performance characteristics determined by ARUP Laboratories. It has not been cleared or approved by the US Food and Drug Administration. This test was performed in a CLIA certified laboratory and is intended for clinical purposes.

Compliance Category

Laboratory Developed Test (LDT)

Note

Hotline History

N/A

CPT Codes

82139

Components

Component Test Code* Component Chart Name LOINC
0080138 Alanine, CSF 22657-1
0080139 Arginine, CSF 22656-3
0080141 Asparagine, CSF 26603-1
0080142 Aspartic Acid, CSF 22655-5
0080143 Citrulline, CSF 22654-8
0080144 Cystine, CSF 22653-0
0080148 Glutamine, CSF 22641-5
0080149 Glutamic Acid, CSF 22652-2
0080151 Glycine, CSF 22650-6
0080152 Histidine, CSF 9453-2
0080153 Homocystine, CSF 22660-5
0080154 Hydroxyproline, CSF 26596-7
0080156 Isoleucine, CSF 22659-7
0080157 Leucine, CSF 9412-8
0080158 Lysine, CSF 22651-4
0080159 Methionine, CSF 22648-0
0080161 Ornithine, CSF 22647-2
0080162 Phenylalanine, CSF 22646-4
0080163 Proline, CSF 22645-6
0080167 Serine, CSF 22644-9
0080168 Taurine, CSF 26614-8
0080169 Threonine, CSF 22643-1
0080171 Tyrosine, CSF 22642-3
0080172 Valine, CSF 22649-8
0080173 Amino Acids, CSF Interpretation 48767-8
2011976 Homocitrulline, CSF 55875-9
2011977 Hydroxylysine, CSF 26595-9
2011983 Sarcosine, CSF 26598-3
2011984 Tryptophan, CSF 26602-3
2011985 Alpha-amino butyric acid, CSF 26586-8
2011986 Allo-isoleucine, CSF 22658-9
2011987 Alpha-aminoadipic acid, CSF 26587-6
2011988 Anserine, CSF 26588-4
2011989 Argininosuccinic Acid, CSF 40838-5
2011990 Beta-alanine, CSF 26589-2
2011991 Beta-amino isobutyric acid, CSF 26590-0
2011992 Cystathionine, CSF 26592-6
2011993 Ethanolamine, CSF 26593-4
2011994 Gamma-amino butyric acid, CSF 26594-2
* Component test codes cannot be used to order tests. The information provided here is not sufficient for interface builds; for a complete test mix, please click the sidebar link to access the Interface Map.

Aliases

  • CSF amino acids
  • quantitative analysis of amino acids
Amino Acids Quantitative by LC-MS/MS, CSF