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