Test Directory

Amino Acids, Quantitative, Plasma

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Updated Test Information:

Test Description
Amino Acids, Quantitative, Plasma
Synonym(s)

Amino Acids, QN Maple Syrup Urine Disease (MSUD), MSUD (Maple Syrup Urine Disease),
Isovaleric Acidemia, Methylmalonic Acidemia (MMA), Mitochondrial Energy Metabolism (Primary Lactic Acidemias),
Nonketotic Hyperglycinemia (NKH), Phenylketonuria (PKU), Propionic Acidemia, Tyrosinemia Type I, Urea Cycle Disorders,
Arginase Deficiency, Argininemia, Argininosuccinic Acid, Argininosuccinic Acid Lyase Deficiency, Argininosuccinic Aciduria,
Carbamoyl Phosphate Synthetase (CPS) Deficiency, Citrullinemia, N-acetyl Glutamate Synthase (NAGS) Deficiency,
NAGS (N-acetyl Glutamate Synthetase), Ornithine Transcarbamylase (OTC) Deficiency, OTC (Ornithine Transcarbamylase),
UCD (Urea Cycle Disorder), Urea Cycle Disorder (AAUCD), Maple syrup disease, Hyperphenylalaninemia, Tyrosinemia type II,
CPS (Carbamoyl Phosphate Synthetase)

Test ID
AAQP
General Information

Useful for:



  • Evaluation of patients with possible inborn errors of metabolism using plasma specimens

  • Evaluation of endocrine disorders, liver diseases, muscle diseases, neoplastic diseases, neurological disorders, nutritional disturbances, renal failure, and burns

Specimen Type

Plasma

Specimen Requirements

  • Preferred: Green Top (Sodium Heparin)

  • Acceptable: Lavender Top (EDTA)

  • Acceptable: Plasma Gel Tube

  • Acceptable: Green Top (Lithium Heparin)

Specimen Collection / Processing Instructions

  • Collect specimen and place on wet ice.

  • Centrifuge immediately or within 4 hours of collection if specimen is kept at refrigerated temperature.

  • Ensure that no buffy coat is transferred, aliquot plasma into a plastic vial and freeze.

Required Information

  • Patient's age is required.

  • Family history

  • Clinical condition (Asymptomatic or acute episode)

  • Diet

  • Drug therapy

Stability

Frozen: 14 days

Limitations

  • Not all patients with homocystinuria will be detected by this assay.

  • If there is a concern for homocystinuria, please order HCYSP / Homocysteine, Total, Plasma in tandem with amino acids.

Methodology

Liquid Chromatography-Tandem Mass Spectrometry (LC-MS/MS)

Estimated TAT

3-5 days

Testing Schedule

Monday-Friday

Test Includes

  • Phosphoserine

  • Phosphoethanolamine

  • Taurine

  • Asparagine

  • Serine

  • Hydroxyproline

  • Glycine

  • Glutamine

  • Aspartic Acid

  • Ethanolamine

  • Histidine

  • Threonine

  • Citrulline

  • Sarcosine

  • Beta-Alanine

  • Alanine

  • Glutamic Acid

  • 1-Methylhistidine

  • 3-Methylhistidine

  • Argininosuccinic Acid

  • Carnosine

  • Anserine

  • Homocitrulline

  • Arginine

  • Alpha-aminoadipic Acid

  • Gamma-amino-n-butyric Acid

  • Beta-aminoisobutyric Acid

  • Alpha-amino-n-butyric Acid

  • Hydroxylysine

  • Proline

  • Ornithine

  • Cystathionine

  • Cystine

  • Lysine

  • Methionine

  • Valine

  • Tyrosine

  • Isoleucine

  • Leucine

  • Phenylalanine

  • Tryptophan

  • Allo-isoleucine

  • Interpretation (AAQP)


 

CPT Code(s)

82139

Additional Information
Minimum Sample Volume

0.3 mL

Reference Range
Reference Values (nmol/mL) < 24 Months 2 - 17 Years ≥ 18 Years
Phosphoserine (PSer) < 109 < 95 < 18
Phosphoethanolamine (PEtN) < 6 < 5 < 12
Taurine (Tau) 37 - 177 38 - 153 42 - 146
Asparagine (Asn) 25 - 91 29 - 87 37 - 92
Serine (Ser) 69 - 271 71 - 208 63 - 187
Hydroxyproline (Hyp) 8 - 61 7 - 35 4 - 29
Glycine (Gly) 111 - 426 149 - 417 126 - 490
Glutamine (Gln) 316 - 1020 329 - 976 371 - 957
Aspartic Acid (Asp) 2 - 20 < 11 < 7
Ethanolamine (EtN) < 70 < 64 < 67
Histidine (His) 10 - 116 12 - 132 39 - 123
Threonine (Thr) 47 - 237 58 - 195 85 - 231
Citrulline (Cit) 9 - 38 11 - 45 17 - 46
Sarcosine (Sar) < 5 < 5 < 5
b-Alanine (bAla) < 28 < 27 < 29
Alanine (Ala) 139 - 474 144 - 557 200 - 579
Glutamic Acid (Glu) 31 - 202 22 - 131 13 - 113
1-Methylhistidine (1MHis) < 11 < 20 < 28
3-Methylhistidine (3MHis) < 1 < 1 2 - 9
Argininosuccinic Acid (Asa) < 2 < 2 < 2
Carnosine (Car) < 13 < 1 < 1
Anserine (Ans) < 1 < 1 < 1
Homocitruline (Hcit) < 5 < 2 < 2
Arginine (Arg) 29 - 134 31 - 132 32 - 120
a-Aminoadipic Acid (Aad) < 4 < 3 < 3
g-Amino-n-butyric Acid (GABA) < 4 < 3 < 2
b-Aminoisobutyric Acid (bAib) < 9 < 5 < 5
a-Amino-n-butyric Acid (Abu) 7 - 28 7 - 31 9 - 37
Hydroxylysine (Hyl) < 4 < 3 < 2
Proline (Pro) 85 - 303 80 - 357 97 - 368
Ornithine (Orn) 20 - 130 22 - 97 38 - 130
Cystathionine (Cth) < 2 < 2 < 5
Cystine (Cys) 2 - 32 2 - 36 3 - 95
Lysine (Lys) 49 - 204 59 - 240 103 - 255
Methionine (Met) 11 - 35 11 - 37 4 - 44
Valine (Val) 83 - 300 106 - 320 136 - 309
Tyrosine (Tyr) 26 - 115 31 - 106 31 - 90
Isoleucine (IIe) 31 - 105 30 - 111 36 - 107
Leucine (Leu) 48 - 175 51 - 196 68 - 183
Phenylalanine (Phe) 28 - 80 30 - 95 35 - 80
Tryptophan (Trp) 17 - 75 23 - 80 29 - 77
Alloisoleucine (Allolle) < 2 < 3 < 5
Performing Lab

Mayo

LOINC Code(s)

20654-0, 26612-2, 20657-3, 20638-3, 20656-5, 20647-4, 20644-1, 20643-3, 20639-1, 26608-0, 20645-8, 20658-1, 20640-9, 26613-0, 26604-9, 20636-7, 20642-5, 20633-4, 20635-9, 32227-1, 26606-4, 26599-1, 55876-7, 20637-5, 26600-7, 26609-8, 26605-6, 20634-2, 266