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HelpTest Code AAQP Amino Acids, Quantitative, Plasma
Reporting Name
Amino Acids, QN, PUseful For
Evaluating patients with possible inborn errors of metabolism using plasma specimens
May aid in evaluation of endocrine disorders, liver diseases, muscle diseases, neoplastic diseases, neurological disorders, nutritional disturbances, kidney failure, and burns
Monitoring of patients treated for various inborn errors of metabolism or other causes of amino acid imbalances
Performing Laboratory
Mayo Clinic Laboratories in Rochester
Specimen Type
PlasmaOrdering Guidance
Body fluids are not acceptable specimens for this test.
For testing urine specimens, order AAPD / Amino Acids, Quantitative, Random, Urine.
For testing spinal fluid specimens, order AACSF / Amino Acids, Quantitative, Spinal Fluid.
Additional Testing Requirements
Not all patients with homocystinuria/homocystinemia will be detected by this assay. For quantitation of total homocysteine, order CMMPP / Cobalamin, Methionine, and Methylmalonic Acid Pathways, Plasma or HCYSP / Homocysteine, Total, Plasma in conjunction with this amino acids profile.
Shipping Instructions
Necessary Information
1. Patient's age is required.
2. Include family history, clinical condition (asymptomatic or acute episode), diet, and drug therapy information.
Specimen Required
Patient Preparation: Patient should fast overnight (4 hours minimum); infants should have specimen collected before next feeding (2-3 hours without total parenteral nutrition if possible).
Supplies: Sarstedt Aliquot Tube, 5 mL (T914)
Collection Container/Tube: Green top (sodium heparin)
Submission Container/Tube: Plastic vial
Specimen Volume: 0.5 mL
Collection Instructions:
1. Collect specimen and place on wet ice. Note: Thrombin-activated tubes should not be used for collection.
2. Centrifuge immediately or within 4 hours of collection if the specimen is kept at refrigerated temperature.
3. Being careful to ensure that no buffy coat is transferred, aliquot plasma into a plastic vial and freeze.
Specimen Minimum Volume
0.3 mL
Specimen Stability Information
| Specimen Type | Temperature | Time | Special Container |
|---|---|---|---|
| Plasma | Frozen | 14 days |
Reference Values
|
Amino acids |
Age groups |
||
|
<24 months |
2-17 years |
≥18 years |
|
|
Taurine (Tau) |
31-354 |
32-181 |
21-123 |
|
Asparagine (Asn) |
18-94 |
25-80 |
23-94 |
|
Serine (Ser) |
59-224 |
53-166 |
55-146 |
|
Hydroxyproline (Hyp) |
<121 |
<73 |
<38 |
|
Glycine (Gly) |
80-500 |
80-500 |
80-500 |
|
Glutamine (Gln) |
356-857 |
353-790 |
447-774 |
|
Aspartic Acid (Asp) |
<48 |
<17 |
<13 |
|
Ethanolamine (EtN) |
<70 |
<30 |
<20 |
|
Histidine (His) |
46-147 |
56-119 |
61-120 |
|
Threonine (Thr) |
49-358 |
48-205 |
73-325 |
|
Citrulline (Cit) |
8-42 |
12-44 |
18-57 |
|
Sarcosine (Sar) |
<20 |
<20 |
<20 |
|
b-Alanine (bAla) |
<36 |
<36 |
<36 |
|
Alanine (Ala) |
139-474 |
144-557 |
200-579 |
|
Glutamic Acid (Glu) |
28-376 |
16-182 |
13-148 |
|
1-Methylhistidine (1MHis) |
<12 |
<12 |
<12 |
|
3-Methylhistidine (3MHis) |
<11 |
<30 |
<35 |
|
Argininosuccinic Acid (Asa) |
<5 |
<5 |
<5 |
|
Homocitruline (Hcit) |
<5 |
<2 |
<2 |
|
Arginine (Arg) |
28-164 |
28-156 |
45-144 |
|
a-Aminoadipic Acid (Aad) |
<4 |
<4 |
<4 |
|
g-Amino-n-butyric Acid (GABA) |
<4 |
<4 |
<4 |
|
b-Aminoisobutyric Acid (bAib) |
<9 |
<5 |
<5 |
|
a-Amino-n-butyric Acid (Abu) |
<40 |
<40 |
<40 |
|
Hydroxylysine (Hyl) |
<4 |
<4 |
<4 |
|
Proline (Pro) |
102-342 |
99-389 |
107-383 |
|
Ornithine (Orn) |
32-171 |
32-148 |
39-154 |
|
Cystathionine (Cth) |
<4 |
<4 |
<4 |
|
Cystine (Cys) |
6-131 |
3-151 |
8-310 |
|
Lysine (Lys) |
83-304 |
61-291 |
105-335 |
|
Methionine (Met) |
12-57 |
13-41 |
13-40 |
|
Valine (Val) |
94-382 |
111-367 |
134-357 |
|
Tyrosine (Tyr) |
27-188 |
36-133 |
36-113 |
|
Isoleucine (IIe) |
23-149 |
26-150 |
29-153 |
|
Leucine (Leu) |
59-213 |
51-216 |
79-217 |
|
Phenylalanine (Phe) |
36-105 |
38-116 |
45-106 |
|
Tryptophan (Trp) |
12-103 |
21-114 |
21-108 |
|
Alloisoleucine (Allolle) |
<5 |
<5 |
<5 |
All results reported in nmol/mL
Day(s) Performed
Monday through Friday
Test Classification
This test was developed and its performance characteristics determined by Mayo Clinic in a manner consistent with CLIA requirements. It has not been cleared or approved by the US Food and Drug Administration.CPT Code Information
82139
LOINC Code Information
| Test ID | Test Order Name | Order LOINC Value |
|---|---|---|
| AAQP | Amino Acids, QN, P | 35083-5 |
| Result ID | Test Result Name | Result LOINC Value |
|---|---|---|
| 3570 | Interpretation (AAQP) | 49247-0 |
| 3512 | Taurine | 20657-3 |
| 3517 | Asparagine | 20638-3 |
| 3516 | Serine | 20656-5 |
| 34458 | Hydroxyproline | 20647-4 |
| 3522 | Glycine | 20644-1 |
| 3518 | Glutamine | 20643-3 |
| 34459 | Aspartic Acid | 20639-1 |
| 34460 | Ethanolamine | 26608-0 |
| 3535 | Histidine | 20645-8 |
| 3515 | Threonine | 20658-1 |
| 3521 | Citrulline | 20640-9 |
| 34461 | Sarcosine | 26613-0 |
| 3532 | Beta-Alanine | 26604-9 |
| 3523 | Alanine | 20636-7 |
| 3520 | Glutamic Acid | 20642-5 |
| 34453 | 1-Methylhistidine | 20633-4 |
| 34454 | 3-Methylhistidine | 20635-9 |
| 32341 | Argininosuccinic Acid | 32227-1 |
| 34456 | Homocitrulline | 55876-7 |
| 3536 | Arginine | 20637-5 |
| 34450 | Alpha-aminoadipic Acid | 26600-7 |
| 34463 | Gamma-amino-n-butyric Acid | 26609-8 |
| 34452 | Beta-aminoisobutyric Acid | 26605-6 |
| 3524 | Alpha-amino-n-butyric Acid | 20634-2 |
| 34464 | Hydroxylysine | 26610-6 |
| 3519 | Proline | 20655-7 |
| 3533 | Ornithine | 20652-4 |
| 34451 | Cystathionine | 26607-2 |
| 3526 | Cystine | 22672-0 |
| 3534 | Lysine | 20650-8 |
| 3527 | Methionine | 20651-6 |
| 3525 | Valine | 20661-5 |
| 3530 | Tyrosine | 20660-7 |
| 3528 | Isoleucine | 20648-2 |
| 3529 | Leucine | 20649-0 |
| 3531 | Phenylalanine | 14875-9 |
| 34465 | Tryptophan | 20659-9 |
| 32347 | Allo-isoleucine | 22670-4 |
Interpretation
When no significant abnormalities are detected, a simple descriptive interpretation is provided. When abnormal results are detected, a detailed interpretation is given, including an overview of the results and their significance, a correlation to available clinical information, elements of differential diagnosis, recommendations for additional biochemical testing, and in vitro confirmatory studies (enzyme assay, molecular analysis), name and phone number of key contacts who may provide these studies, and a phone number to reach one of the laboratory directors in case the referring physician has additional questions.
Clinical Reference
1. Part 8: Amino Acids. In: Valle DL, Antonarakis S, Ballabio A, Beaudet AL, Mitchell GA. eds. The Online Metabolic and Molecular Bases of Inherited Disease. McGraw-Hill, 2019. Accessed October 24, 2024 Available at https://ommbid.mhmedical.com/book.aspx?bookID=2709#225069340
2. Pasquali M, Longo N: Amino acids. In: Blau N, Dionisi Vici C, Ferreira CR, Vianey-Saban C, van Karnebeek CDM, eds. Physician's Guide to the Diagnosis, Treatment and Follow-up of Inherited Metabolic Diseases. 2nd ed. Springer-Verlag; 2022:41-50
Report Available
3 to 5 daysTesting Algorithm
Testing includes quantitation of the following amino acids: taurine, threonine, serine, asparagine, glutamic acid, glutamine, proline, alanine, citrulline, alpha-amino-n-butyric acid, valine, cystine, methionine, isoleucine, leucine, tyrosine, phenylalanine, beta-alanine, ornithine, lysine, histidine, argininosuccinic acid, allo-isoleucine, arginine, hydroxyproline, glycine, aspartic acid, ethanolamine, sarcosine, 1-methylhistidine, 3-methylhistidine, homocitrulline, alpha-aminoadipic acid, gamma-amino-n-butyric acid, beta-aminoisobutyric acid, hydroxylysine, cystathionine, and tryptophan.
For more information see Epilepsy: Unexplained Refractory and/or Familial Testing Algorithm.
Method Name
Liquid Chromatography Tandem Mass Spectrometry (LC-MS/MS)
Portions of this test are covered by patents held by Quest Diagnostics
Special Instructions
Forms
If not ordering electronically, complete, print, and send a Biochemical Genetics Test Request (T798) with the specimen.
Aliases:
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)