| Test Description |
Amino Acids, Quantitative, Plasma
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| 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) |
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| Test ID |
AAQP
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| 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
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| Specimen Type |
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| Specimen Requirements |
- Preferred: Green Top (Sodium Heparin)
- Acceptable: Lavender Top (EDTA)
- Acceptable: Plasma Gel Tube
- Acceptable: Green Top (Lithium Heparin)
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| 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.
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| Minimum Sample Volume |
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| Required Information |
- Patient's age is required.
- Family history
- Clinical condition (Asymptomatic or acute episode)
- Diet
- Drug therapy
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| Stability |
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| 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.
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| Methodology |
Liquid Chromatography-Tandem Mass Spectrometry (LC-MS/MS) |
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| Estimated TAT |
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| Testing Schedule |
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| 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)
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| CPT Code(s) |
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| 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 |
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| Performing Lab |
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| 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 |
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| Additional Information |
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