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.
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 https://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, Plasma Less than or equal to 4 µmol/L
  Alpha-amino butyric acid, Plasma Less than or equal to 40 µmol/L
  Alanine, Plasma
Age Reference Interval
0-30 days 140-480  µmol/L
1 month-11 months 150-520  µmol/L
1 year and older 160-530  µmol/L

  Allo-isoleucine, Plasma Less than or equal to 5 µmol/L
  Anserine, Plasma Less than or equal to 5 µmol/L
  Arginine, Plasma
Age Reference Interval
0-30 days 16-140  µmol/L
1 month-11 months 35-140  µmol/L
1 year and older 35-125  µmol/L

  Argininosuccinic Acid, Plasma Less than or equal to 2 µmol/L
  Asparagine, Plasma 20-80 µmol/L
  Aspartic Acid, Plasma
Age Reference Interval
0-30 days Less than or equal to 45 µmol/L
1 month-11 months Less than or equal to 30 µmol/L
1 year and older Less than or equal to 15 µmol/L

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

  Citrulline, Plasma
Age Reference Interval
0 day to 11 months 7-40 µmol/L
1 year and older 10-45 µmol/L

  Cystathionine, Plasma Less than or equal to 5 µmol/L
  Cystine, Plasma
Age
Reference Interval
0-30 days 10-60 µmol/L
1 month-11 months 10-50 µmol/L
1 year and older 10-65 µmol/L

  Ethanolamine, Plasma
Age Reference Interval
0-30 days Less than or equal to 100 µmol/L
1 month-11 months Less than or equal to 25 µmol/L
1 year and older Less than or equal to 15 µmol/L

  Gamma-amino butyric acid, Plasma Less than or equal to 5 µmol/L
  Glutamic Acid, Plasma
Age Reference Range
0-30 days 30-240 µmol/L
1 month-11 months 30-210 µmol/L
1 year and older 15-130 µmol/L

  Glutamine, Plasma
Age Reference Interval
0-30 days 295-900 µmol/L
1 month-11 months 400-850 µmol/L
1 year and older 380-680 µmol/L

  Glycine, Plasma
Age Reference Interval
0-30 days 160-470 µmol/L
1 month-11 months 120-375 µmol/L
1 year and older 140-420 µmol/L

  Histidine, Plasma 50-130 µmol/L
  Homocitrulline, Plasma Less than or equal to 5 µmol/L
  Homocystine, Plasma Less than or equal to 2 µmol/L
  Hydroxylysine, Plasma Less than or equal to 5 µmol/L
  Hydroxyproline, Plasma
Age Reference Interval
0-30 days 15-90 µmol/L
1 month-11 months 10-70 µmol/L
1 year and older 5-40 µmol/L

  Isoleucine, Plasma
Age Reference Interval
0-30 days 20-110 µmol/L
1 month and older 30-120 µmol/L

  Leucine, Plasma
Age Reference Interval
0-11 months 50-180 µmol/L
1 year and older 60-180 µmol/L

  Lysine, Plasma
Age Reference Interval
0-30 days 70-270 µmol/L
1 month-11 months 80-260 µmol/L
1 year and older 85-230 µmol/L

  Methionine, Plasma
Age Reference Interval
0-11 months 15-55 µmol/L
1 year and older 15-40 µmol/L

  Ornithine, Plasma
Age Reference Interval
0-30 days 30-180 µmol/L
1 month-11 months 30-140 µmol/L
1 year and older 25-110 µmol/L

  Phenylalanine, Plasma
Age Reference Interval
0-30 days 30-95 µmol/L
1 month-11 months 30-90 µmol/L
1 year and older 30-82 µmol/L

  Proline, Plasma
Age Reference Interval
0-30 days 110-340 µmol/L
1 month-11 months 100-320 µmol/L
1 year and older 90-350 µmol/L

  Sarcosine, Plasma Less than or equal to 5 µmol/L
  Serine, Plasma
Age Reference Interval
0-30 days 90-340 µmol/L
1 month-11 months 90-275 µmol/L
1 year and older 60-170 µmol/L

  Taurine, Plasma
Age Reference Interval
0-30 days 30-250 µmol/L
1 month-11 months 30-170 µmol/L
1 year and older 30 -130 µmol/L

  Threonine, Plasma
Age Reference Interval
0-30 days 60-400 µmol/L
1 month-11 months 60-310 µmol/L
1 year and older 60-190 µmol/L

  Tryptophan, Plasma
Age Reference Interval
0-30 days 15-75 µmol/L
1 month-11 months 20-85 µmol/L
1 year and older 25-80 µmol/L

  Tyrosine, Plasma
Age Reference Interval
0-30 days 30-140 µmol/L
1 month-11 months 30-130 µmol/L
1 year and older 35-110 µmol/L

  Valine, Plasma
Age Reference Interval
0-30 days 80-270 µmol/L
1 month-11 months 90-310 µmol/L
1 year and older 120-320 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
N/A
CPT Codes

82139

Components
Component Test Code* Component Chart Name LOINC
0080010 Alanine, Plasma 20636-7
0080025 Arginine, Plasma 20637-5
0080035 Aspartic Acid, Plasma 20639-1
0080083 Citrulline, Plasma 20640-9
0080140 Glutamine, Plasma 20643-3
0080150 Glutamic Acid, Plasma 20642-5
0080155 Glycine, Plasma 20644-1
0080160 Histidine, Plasma 20645-8
0080170 Hydroxyproline, Plasma 20647-4
0080185 Isoleucine, Plasma 20648-2
0080205 Leucine, Plasma 20649-0
0080210 Lysine, Plasma 20650-8
0080225 Methionine, Plasma 20651-6
0080245 Ornithine, Plasma 20652-4
0080265 Phenylalanine, Plasma 14875-9
0080295 Proline, Plasma 20655-7
0080335 Serine, Plasma 20656-5
0080345 Taurine, Plasma 20657-3
0080350 Threonine, Plasma 20658-1
0080356 Tyrosine, Plasma 20660-7
0080370 Valine, Plasma 20661-5
0080715 Homocystine, Plasma 20646-6
0080717 Allo-isoleucine, Plasma 22670-4
0080953 Amino Acids, Plasma Interpretation 13500-4
0099865 Cystine, Plasma 22672-0
2009392 Alpha-amino butyric acid, Plasma 20634-2
2009393 Alpha-aminoadipic acid, Plasma 26600-7
2009394 Argininosuccinic Acid, Plasma 32227-1
2009395 Beta-amino isobutyric acid, Plasma 26605-6
2009396 Beta-alanine, Plasma 26604-9
2009398 Ethanolamine, Plasma 26608-0
2009399 Gamma-amino butyric acid, Plasma 26609-8
2009402 Sarcosine, Plasma 26613-0
2009403 Tryptophan, Plasma 20659-9
2009404 Anserine, Plasma 26599-1
2009405 Asparagine, Plasma 20638-3
2009406 Cystathionine, Plasma 26607-2
2009407 Homocitrulline, Plasma 55876-7
2009408 Hydroxylysine, Plasma 26610-6
* 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
Amino Acids Quantitative by LC-MS/MS, Plasma