Test Id : AAQP
Amino Acids, Quantitative, Plasma
Useful For
Suggests clinical disorders or settings where the test may be helpful
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
Testing Algorithm
Delineates situations when tests are added to the initial order. This includes reflex and additional tests.
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
A short description of the method used to perform the test
Liquid Chromatography Tandem Mass Spectrometry (LC-MS/MS)
Portions of this test are covered by patents held by Quest Diagnostics
NY State Available
Indicates the status of NY State approval and if the test is orderable for NY State clients.
Reporting Name
Lists a shorter or abbreviated version of the Published Name for a test
Aliases
Lists additional common names for a test, as an aid in searching
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)
Testing Algorithm
Delineates situations when tests are added to the initial order. This includes reflex and additional tests.
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.
Specimen Type
Describes the specimen type validated for testing
Plasma
Ordering 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.
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
Defines the optimal specimen required to perform the test and the preferred volume to complete testing
Patient Preparation: Patient should fast a minimum of 4 hours; 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.
Special Instructions
Library of PDFs including pertinent information and forms related to the test
Forms
If not ordering electronically, complete, print, and send a Biochemical Genetics Test Request (T798) with the specimen.
Specimen Minimum Volume
Defines the amount of sample necessary to provide a clinically relevant result as determined by the testing laboratory. The minimum volume is sufficient for one attempt at testing.
0.3 mL
Reject Due To
Identifies specimen types and conditions that may cause the specimen to be rejected
| Gross hemolysis | OK |
| Gross lipemia | OK |
| Gross icterus | OK |
Specimen Stability Information
Provides a description of the temperatures required to transport a specimen to the performing laboratory, alternate acceptable temperatures are also included
| Specimen Type | Temperature | Time | Special Container |
|---|---|---|---|
| Plasma | Frozen | 14 days |
Useful For
Suggests clinical disorders or settings where the test may be helpful
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
Testing Algorithm
Delineates situations when tests are added to the initial order. This includes reflex and additional tests.
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.
Clinical Information
Discusses physiology, pathophysiology, and general clinical aspects, as they relate to a laboratory test
Amino acids are the basic structural units that comprise proteins and are found throughout the body. Many inborn errors of amino acid metabolism, such as phenylketonuria and tyrosinemia, have been identified. Amino acid disorders can manifest at any age, but most become evident in infancy or early childhood. These disorders result in the accumulation or the deficiency of 1 or more amino acids in biological fluids, which leads to the clinical signs and symptoms of the specific amino acid disorder.
The clinical presentation is dependent upon the specific amino acid disorder. In general, affected patients may experience failure to thrive, neurologic symptoms, digestive problems, dermatologic findings, and physical and cognitive delays. If not diagnosed and treated promptly, amino acid disorders can result in intellectual disabilities and, possibly, death.
Treatment for amino acid disorders includes very specific dietary modifications. Nonessential amino acids are synthesized by the body, while essential amino acids are not and must be obtained through an individual's diet. Therapeutic diets are coordinated and closely monitored by a dietician or physician. They are structured to provide the necessary balance of amino acids with particular attention to essential amino acids and those that are abnormal in a particular disorder. Patients must pay close attention to the protein content in their diet and generally need to supplement with medical formulas and foods. Dietary compliance is monitored by periodic analysis of plasma amino acids.
In addition, plasma amino acid analysis may have clinical importance in the evaluation of several acquired conditions, including endocrine disorders, liver diseases, muscle diseases, neoplastic diseases, neurological disorders, nutritional disturbances, kidney failure, and burns.
Reference Values
Describes reference intervals and additional information for interpretation of test results. May include intervals based on age and sex when appropriate. Intervals are Mayo-derived, unless otherwise designated. If an interpretive report is provided, the reference value field will state this.
| Amino acids | Age groups | ||
| <24 months | 2-17 years | > or =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
Interpretation
Provides information to assist in interpretation of the test results
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.
Cautions
Discusses conditions that may cause diagnostic confusion, including improper specimen collection and handling, inappropriate test selection, and interfering substances
Reference values are for fasting patients.
Patients with phenylketonuria (PKU) who are treated with pegvaliase-pqpz (Palynziq) may have false low phenylalanine concentrations due to persistent activity in collected blood specimens.
This assay does not measure total homocysteine. If relevant disorders are considered, also order CMMPP / Cobalamin, Methionine, and Methylmalonic Acid Pathways, Plasma or HCYSP / Homocysteine, Total, Plasma analysis.
Clinical Reference
Recommendations for in-depth reading of a clinical nature
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
Method Description
Describes how the test is performed and provides a method-specific reference
Quantitative analysis of amino acids is performed by liquid chromatography tandem mass spectrometry (LC-MS/MS). Patient samples are combined with isotopically labeled internal standard. Following protein precipitation, the supernatant is subjected to hydrophilic interaction liquid chromatography for the separation of isomers with MS/MS detection of the underivatized amino acids.(Unpublished Mayo method)
PDF Report
Indicates whether the report includes an additional document with charts, images or other enriched information
Day(s) Performed
Outlines the days the test is performed. This field reflects the day that the sample must be in the testing laboratory to begin the testing process and includes any specimen preparation and processing time before the test is performed. Some tests are listed as continuously performed, which means that assays are performed multiple times during the day.
Monday through Friday
Report Available
The interval of time (receipt of sample at Mayo Clinic Laboratories to results available) taking into account standard setup days and weekends. The first day is the time that it typically takes for a result to be available. The last day is the time it might take, accounting for any necessary repeated testing.
Specimen Retention Time
Outlines the length of time after testing that a specimen is kept in the laboratory before it is discarded
Performing Laboratory Location
Indicates the location of the laboratory that performs the test
Fees :
Several factors determine the fee charged to perform a test. Contact your U.S. or International Regional Manager for information about establishing a fee schedule or to learn more about resources to optimize test selection.
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- Prospective clients should contact their account representative. For assistance, contact Customer Service.
Test Classification
Provides information regarding the medical device classification for laboratory test kits and reagents. Tests may be classified as cleared or approved by the US Food and Drug Administration (FDA) and used per manufacturer instructions, or as products that do not undergo full FDA review and approval, and are then labeled as an Analyte Specific Reagent (ASR) product.
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
Provides guidance in determining the appropriate Current Procedural Terminology (CPT) code(s) information for each test or profile. The listed CPT codes reflect Mayo Clinic Laboratories interpretation of CPT coding requirements. It is the responsibility of each laboratory to determine correct CPT codes to use for billing.
CPT codes are provided by the performing laboratory.
CPT codes are provided by the performing laboratory.
82139
LOINC® Information
Provides guidance in determining the Logical Observation Identifiers Names and Codes (LOINC) values for the order and results codes of this test. LOINC values are provided by the performing laboratory.
| Test Id | Test Order Name | Order LOINC Value |
|---|---|---|
| AAQP | Amino Acids, QN, P | 35083-5 |
| Result Id | Test Result Name |
Result LOINC Value
Applies only to results expressed in units of measure originally reported by the performing laboratory. These values do not apply to results that are converted to other units of measure.
|
|---|---|---|
| 3512 | Taurine | 20657-3 |
| 3517 | Asparagine | 20638-3 |
| 3516 | Serine | 20656-5 |
| 3522 | Glycine | 20644-1 |
| 3518 | Glutamine | 20643-3 |
| 3535 | Histidine | 20645-8 |
| 3515 | Threonine | 20658-1 |
| 3521 | Citrulline | 20640-9 |
| 3532 | Beta-Alanine | 26604-9 |
| 3523 | Alanine | 20636-7 |
| 3520 | Glutamic Acid | 20642-5 |
| 32341 | Argininosuccinic Acid | 32227-1 |
| 3536 | Arginine | 20637-5 |
| 3524 | Alpha-amino-n-butyric Acid | 20634-2 |
| 3519 | Proline | 20655-7 |
| 3533 | Ornithine | 20652-4 |
| 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 |
| 3570 | Interpretation (AAQP) | 49247-0 |
| 32347 | Allo-isoleucine | 22670-4 |
| 34450 | Alpha-aminoadipic Acid | 26600-7 |
| 34451 | Cystathionine | 26607-2 |
| 34452 | Beta-aminoisobutyric Acid | 26605-6 |
| 34453 | 1-Methylhistidine | 20633-4 |
| 34454 | 3-Methylhistidine | 20635-9 |
| 34456 | Homocitrulline | 55876-7 |
| 34458 | Hydroxyproline | 20647-4 |
| 34459 | Aspartic Acid | 20639-1 |
| 34460 | Ethanolamine | 26608-0 |
| 34461 | Sarcosine | 26613-0 |
| 34463 | Gamma-amino-n-butyric Acid | 26609-8 |
| 34464 | Hydroxylysine | 26610-6 |
| 34465 | Tryptophan | 20659-9 |