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Amino Acids Quantitative by LC-MS/MS, Plasma
2009389
Ordering Recommendation
Diagnose and monitor aminoacidopathies (eg, PKU, MSUD).
Mnemonic
AA QNT P
Methodology
Quantitative Liquid Chromatography/Tandem Mass Spectrometry
Performed
Mon-Fri
Reported
2-5 days
New York DOH Approval Status
This test is New York DOH approved.
ARUP Consult®
Disease Topics
Specimen Required
Patient Preparation
Adults: Fasting specimen preferred. Infants and children: Draw specimen prior to feeding or 2-3 hours after a meal.  
Collect
Green (sodium or lithium heparin).  
Specimen Preparation
Separate plasma from cells ASAP or within 2 hours of collection. Avoid transferring buffy coat material. Transfer 0.5 mL plasma to an ARUP Standard Transport Tube and freeze immediately. (Min: 0.25 mL)  
Storage/Transport Temperature
CRITICAL FROZEN. Separate specimens must be submitted when multiple tests are ordered.  
Unacceptable Conditions
Hemolyzed specimens.  
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 http://www.aruplab.com/patienthistory or by contacting ARUP Client Services.  
Stability
After separation from cells: Ambient: Unacceptable; Refrigerated: 24 hours; Frozen: 1 month  
Reference Interval
Test Number
Components
Reference Interval
 Alpha-aminoadipic acid, Plasma
0-30 days1 month-11 months1 year and older
0 - 4 µmol/L0 - 3 µmol/L0 - 3 µmol/L

 Alpha-amino butyric acid, Plasma
0-30 days1 month-11 months1 year and older
0 - 40 µmol/L0 - 40 µmol/L0 - 30 µmol/L

 Alanine, Plasma
0-30 days
1 month-11 months
1 year and older
130 - 560 µmol/L150 - 570 µmol/L150 - 570 µmol/L

 Allo-isoleucine, Plasma
0-30 days1 month-11 months1 year and older
0 - 5 µmol/L0 - 3 µmol/L0 - 3 µmol/L

 Anserine, Plasma
0-30 days1 month-11 months1 year and older
0 - 2 µmol/L0 - 2 µmol/L0 - 2 µmol/L

 Arginine, Plasma
0-30 days1 month-11 months1 year and older
15 - 160 µmol/L20 -160 µmol/L40 - 160 µmol/L

 Argininosuccinic Acid, Plasma
0-30 days1 month-11 months1 year and older
0 - 2 µmol/L0 - 2 µmol/L0 - 2 µmol/L

 Asparagine, Plasma
0-30 days1 month-11 months1 year and older
20 - 80 µmol/L30 - 80 µmol/L30 - 80 µmol/L

 Aspartic Acid, Plasma
0-30 days1 month-11 months1 year and older
5 - 50 µmol/L0 - 40 µmol/L0 - 25 µmol/L

 Beta-alanine, Plasma
0-30 days1 month-11 months1 year and older
0 - 20 µmol/L0 - 20 µmol/L0 - 20 µmol/L

 Beta-amino isobutyric acid, Plasma
0-30 days1 month-11 months1 year and older
0 -10 µmol/L0 -10 µmol/L0 - 5 µmol/L

 Citrulline, Plasma
0-30 days1 month-11 months1 year and older
5-50 µmol/L5-50 µmol/L10-60 µmol/L

 Cystathionine, Plasma
0-30 days1 month-11 months1 year and older
0 -10 µmol/L0 - 5 µmol/L0 - 5 µmol/L

 Cystine, Plasma
0-30 days1 month-11 months1 year and older
5-50 µmol/L7-50 µmol/L7-70 µmol/L

 Ethanolamine, Plasma
0-30 days1 month-11 months1 year and older
0 - 40 µmol/L0 - 20 µmol/L0 - 15 µmol/L

 Gamma-amino butyric acid, Plasma
0-30 days1 month-11 months1 year and older
0 - 2 µmol/L0 - 2 µmol/L0 - 2 µmol/L

 Glutamic Acid, Plasma
0-30 days1 month-11 months1 year and older
50 - 300 µmol/L25 - 200 µmol/L10 - 120 µmol/L

 Glutamine, Plasma
0-30 days1 month-11 months1 year and older
285 - 950 µmol/L410 - 900 µmol/L410 - 700 µmol/L

 Glycine, Plasma
0-30 days1 month-11 months1 year and older
150 - 500 µmol/L120 - 450 µmol/L120 - 450 µmol/L

 Histidine, Plasma
0-30 days1 month-11 months1 year and older
50 - 110 µmol/L50 - 110 µmol/L50 - 110 µmol/L

 Homocitrulline, Plasma
0-30 days1 month-11 months1 year and older
0 - 5 µmol/L0 - 3 µmol/L0 - 2 µmol/L

 Homocystine, Plasma
0-30 days1 month-11 months1 year and older
0 - 2 µmol/L0 - 2 µmol/L0 - 2 µmol/L

 Hydroxylysine, Plasma
0-30 days1 month-11 months1 year and older
0 - 5 µmol/L0 - 5 µmol/L0 - 5 µmol/L

 Hydroxyproline, Plasma
0-30 days1 month-11 months1 year and older
0 - 100 µmol/L0 - 90 µmol/L0 - 55 µmol/L

 Isoleucine, Plasma
0-30 days1 month-11 months1 year and older
20 - 130 µmol/L20 - 130 µmol/L30 - 130 µmol/L

 Leucine, Plasma
0-30 days1 month-11 months1 year and older
50 - 200 µmol/L50 - 200 µmol/L60 - 230 µmol/L

 Lysine, Plasma
0-30 days1 month-11 months1 year and older
60 - 300 µmol/L60 - 280 µmol/L80 - 250 µmol/L

 Methionine, Plasma
0-30 days1 month-11 months1 year and older
10 - 60 µmol/L10 - 60 µmol/L14 - 50 µmol/L

 Ornithine, Plasma
0-30 days1 month-11 months1 year and older
30 - 200 µmol/L20 - 160 µmol/L20 - 135 µmol/L

 Phenylalanine, Plasma
0-30 days1 month-11 months1 year and older
30 - 100 µmol/L30 - 90 µmol/L30 - 80 µmol/L

 Proline, Plasma
0-30 days1 month-11 months1 year and older
100-400 µmol/L80-400 µmol/L80-400 µmol/L

 Sarcosine, Plasma
0-30 days1 month-11 months1 year and older
0 - 5 µmol/L0 - 5 µmol/L0 - 5 µmol/L

 Serine, Plasma
0-30 days1 month-11 months1 year and older
90 - 300 µmol/L90 - 300 µmol/L60 - 200 µmol/L

 Taurine, Plasma
0-30 days1 month-11 months1 year and older
25 - 300 µmol/L25 - 220 µmol/L25 -150 µmol/L

 Threonine, Plasma
0-30 days1 month-11 months1 year and older
50 - 400 µmol/L50 - 300 µmol/L60 - 200 µmol/L

 Tryptophan, Plasma
0-30 days1 month-11 months1 year and older
15 - 90 µmol/L15 - 90 µmol/L30 - 100 µmol/L

 Tyrosine, Plasma
0-30 days1 month-11 months1 year and older
30 - 140 µmol/L30 - 140 µmol/L30 - 120 µmol/L

 Valine, Plasma
0-30 days1 month-11 months1 year and older
80 - 300 µmol/L80 - 300 µmol/L100 - 300 µmol/L


Interpretive Data


Compliance Statement B: This test was developed and its performance characteristics determined by ARUP Laboratories. The U.S. Food and Drug Administration has not approved or cleared this test; however, FDA clearance or approval is not currently required for clinical use. The results are not intended to be used as the sole means for clinical diagnosis or patient management decisions.
Note
Common indications for amino acid testing include clinical situations such as: 1) acute life-threatening episode, 2) failure to thrive, 3) recurrent vomiting, 4) neurological deterioration, 5) hyperammonemia, 6) lethargy, 7) metabolic acidosis, and 8) testing or following therapy for a specific inborn error of metabolism (PKU, MSUD, tyrosinemia, etc.). Listing of clinical information is particularly important for appropriate interpretation.
CPT Code(s)
82139
Components
Component Test Code*Component Chart NameLOINC
0080010Alanine, Plasma20636-7
0080025Arginine, Plasma20637-5
0080035Aspartic Acid, Plasma20639-1
0080083Citrulline, Plasma20640-9
0080140Glutamine, Plasma20643-3
0080150Glutamic Acid, Plasma20642-5
0080155Glycine, Plasma20644-1
0080160Histidine, Plasma20645-8
0080170Hydroxyproline, Plasma20647-4
0080185Isoleucine, Plasma20648-2
0080205Leucine, Plasma20649-0
0080210Lysine, Plasma20650-8
0080225Methionine, Plasma20651-6
0080245Ornithine, Plasma20652-4
0080265Phenylalanine, Plasma14875-9
0080295Proline, Plasma20655-7
0080335Serine, Plasma20656-5
0080345Taurine, Plasma20657-3
0080350Threonine, Plasma20658-1
0080356Tyrosine, Plasma20660-7
0080370Valine, Plasma20661-5
0080715Homocystine, Plasma20646-6
0080717Allo-isoleucine, Plasma22670-4
0080953Amino Acids, Plasma Interpretation13500-4
0099865Cystine, Plasma22672-0
2009392Alpha-amino butyric acid, Plasma20634-2
2009393Alpha-aminoadipic acid, Plasma26600-7
2009394Argininosuccinic Acid, Plasma32227-1
2009395Beta-amino isobutyric acid, Plasma26605-6
2009396Beta-alanine, Plasma26604-9
2009398Ethanolamine, Plasma26608-0
2009399Gamma-amino butyric acid, Plasma26609-8
2009402Sarcosine, Plasma26613-0
2009403Tryptophan, Plasma20659-9
2009404Anserine, Plasma26599-1
2009405Asparagine, Plasma20638-3
2009406Cystathionine, Plasma26607-2
2009407Homocitrulline, Plasma55876-7
2009408Hydroxylysine, Plasma26610-6
* 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
  • Allo-Isoleucine  Amino Acids, Maple Syrup Urine Disease Panel, Plasma
  • Argininosuccinic Acid (ASA)
  • Branched Chain Amino Acids
  • Cystine
  • Free Homocystine
  • Glycine
  • Isoleucine
  • Isovaleric Acidemia
  • Leucine
  • Methylmalonic Acidemia (MMA)
  • Mitochondrial Energy Metabolism (Primary Lactic Acidemias)
  • Nonketotic Hyperglycinemia (NKH)
  • Phenylketonuria (PKU)
  • Propionic Acidemia
  • Quantitative Plasma Amino
  • Valine