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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
Effective November 18, 2019
Test Number
Components
Reference Interval
 Alpha-aminoadipic acid, PlasmaLess than or equal to 4 µmol/L
 Alpha-amino butyric acid, PlasmaLess than or equal to 40 µmol/L
 Alanine, Plasma
AgeReference Interval
0-30 days140-480  µmol/L
1 month-11 months150-520  µmol/L
1 year and older160-530  µmol/L

 Allo-isoleucine, PlasmaLess than or equal to 5 µmol/L
 Anserine, PlasmaLess than or equal to 5 µmol/L
 Arginine, Plasma
AgeReference Interval
0-30 days16-140  µmol/L
1 month-11 months35-140  µmol/L
1 year and older35-125  µmol/L

 Argininosuccinic Acid, PlasmaLess than or equal to 2 µmol/L
 Asparagine, Plasma20-80 µmol/L
 Aspartic Acid, Plasma
AgeReference Interval
0-30 daysLess than or equal to 45 µmol/L
1 month-11 monthsLess than or equal to 30 µmol/L
1 year and olderLess than or equal to 15 µmol/L

 Beta-alanine, PlasmaLess than or equal to 25 µmol/L
 Beta-amino isobutyric acid, Plasma
Age
Reference Interval
0 day to 11 monthsLess than or equal to 15 µmol/L
1 year and olderLess than or equal to 10 µmol/L

 Citrulline, Plasma
AgeReference Interval
0 day to 11 months7-40 µmol/L
1 year and older10-45 µmol/L

 Cystathionine, PlasmaLess than or equal to 5 µmol/L
 Cystine, Plasma
Age
Reference Interval
0-30 days10-60 µmol/L
1 month-11 months10-50 µmol/L
1 year and older10-65 µmol/L

 Ethanolamine, Plasma
AgeReference Interval
0-30 daysLess than or equal to 100 µmol/L
1 month-11 monthsLess than or equal to 25 µmol/L
1 year and olderLess than or equal to 15 µmol/L

 Gamma-amino butyric acid, PlasmaLess than or equal to 5 µmol/L
 Glutamic Acid, Plasma
AgeReference Range
0-30 days30-240 µmol/L
1 month-11 months30-210 µmol/L
1 year and older15-130 µmol/L

 Glutamine, Plasma
AgeReference Interval
0-30 days295-900 µmol/L
1 month-11 months400-850 µmol/L
1 year and older380-680 µmol/L

 Glycine, Plasma
AgeReference Interval
0-30 days160-470 µmol/L
1 month-11 months120-375 µmol/L
1 year and older140-420 µmol/L

 Histidine, Plasma50-130 µmol/L
 Homocitrulline, PlasmaLess than or equal to 5 µmol/L
 Homocystine, PlasmaLess than or equal to 2 µmol/L
 Hydroxylysine, PlasmaLess than or equal to 5 µmol/L
 Hydroxyproline, Plasma
AgeReference Interval
0-30 days15-90 µmol/L
1 month-11 months10-70 µmol/L
1 year and older5-40 µmol/L

 Isoleucine, Plasma
AgeReference Interval
0-30 days20-110 µmol/L
1 month and older30-120 µmol/L

 Leucine, Plasma
AgeReference Interval
0-11 months50-180 µmol/L
1 year and older60-180 µmol/L

 Lysine, Plasma
AgeReference Interval
0-30 days70-270 µmol/L
1 month-11 months80-260 µmol/L
1 year and older85-230 µmol/L

 Methionine, Plasma
AgeReference Interval
0-11 months15-55 µmol/L
1 year and older15-40 µmol/L

 Ornithine, Plasma
AgeReference Interval
0-30 days30-180 µmol/L
1 month-11 months30-140 µmol/L
1 year and older25-110 µmol/L

 Phenylalanine, Plasma
AgeReference Interval
0-30 days30-95 µmol/L
1 month-11 months30-90 µmol/L
1 year and older30-82 µmol/L

 Proline, Plasma
AgeReference Interval
0-30 days110-340 µmol/L
1 month-11 months100-320 µmol/L
1 year and older90-350 µmol/L

 Sarcosine, PlasmaLess than or equal to 5 µmol/L
 Serine, Plasma
AgeReference Interval
0-30 days90-340 µmol/L
1 month-11 months90-275 µmol/L
1 year and older60-170 µmol/L

 Taurine, Plasma
AgeReference Interval
0-30 days30-250 µmol/L
1 month-11 months30-170 µmol/L
1 year and older30 -130 µmol/L

 Threonine, Plasma
AgeReference Interval
0-30 days60-400 µmol/L
1 month-11 months60-310 µmol/L
1 year and older60-190 µmol/L

 Tryptophan, Plasma
AgeReference Interval
0-30 days15-75 µmol/L
1 month-11 months20-85 µmol/L
1 year and older25-80 µmol/L

 Tyrosine, Plasma
AgeReference Interval
0-30 days30-140 µmol/L
1 month-11 months30-130 µmol/L
1 year and older35-110 µmol/L

 Valine, Plasma
AgeReference Interval
0-30 days80-270 µmol/L
1 month-11 months90-310 µmol/L
1 year and older120-320 µmol/L


Interpretive Data


Compliance Statement B: For laboratory developed tests not using a RUO kit, and for FDA approved, cleared or 510(k) exempt assays with alterations. 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.
Hotline History
View Hotline History
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