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22224 Amino Acids, Quantitative Panel (AAQP)

Amino Acids, Quantitative Panel (AAQP)
Test Code: AAIEPSO
Synonyms/Keywords
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 Components
​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, phosphoserine, phosphoethanolamine, hydroxyproline, glycine, aspartic acid, ethanolamine, sarcosine, 1-methylhistidine, 3-methylhistidine, carnosine, anserine, homocitruline, alpha-aminoadipic acid, gamma-amino-n-butyric acid, beta-aminoisobutyric acid, hydroxylysine, cystathionine, and tryptophan.
Useful For
​Evaluation of 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, renal failure, and burns
Specimen Requirements
Fasting Required Specimen Type Preferred Container/Tube Acceptable Container/Tube Specimen Volume Specimen Minimum Volume
(allows for 1 repeat)
Pediatric Minimum Volume
(no repeat)
​Yes Plasma​ Sodium-heparin Green Top Tube (GTT)​ EDTA Lavender Top Tube (LTT), Lithium-heparin Plasma Separator Tube (PST), Lithium-heparin Green Top Tube (GTT)​ 0.5 mL​ 0.3 mL​
Collection Processing Instructions

1. Patient's age is required.

2. Include family history, clinical condition (asymptomatic or acute episode), diet, and drug therapy information.

Patient Preparation: Fasting (overnight preferred, 4 hours minimum). Infants should be drawn just before next feeding (2-3 hours without total parenteral nutrition, if possible).

Collection Instructions:

1. Collect specimen and place on wet ice.

2. Centrifuge immediately or within 4 hours of collection if 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. 

Send plasma frozen. 

Specimen Stability Information
Specimen Type Temperature Time
Plasma ​Frozen ​14 days
Interference

​Reference values are for fasting patients.

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. 

Performing Laboratory Information
Performing Location Day(s) Test Performed Analytical Time Methodology/Instrumentation
Mayo Clinic Laboratories
Monday through Friday​
3 to 5 days​
Liquid Chromatography-Tandem Mass Spectrometry (LC-MS/MS)​
Reference Lab
Reference Range Information
See Mayo Clinic Laboratories for age specific reference ranges. 
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 of 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.
Outreach CPTs
CPT Modifier
(if needed)
Quantity Description Comments
​82139
Synonyms/Keywords
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 Components
​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, phosphoserine, phosphoethanolamine, hydroxyproline, glycine, aspartic acid, ethanolamine, sarcosine, 1-methylhistidine, 3-methylhistidine, carnosine, anserine, homocitruline, alpha-aminoadipic acid, gamma-amino-n-butyric acid, beta-aminoisobutyric acid, hydroxylysine, cystathionine, and tryptophan.
Ordering Applications
Ordering Application Description
​Centricity ​Amino Acids Panel (AAQP)
If the ordering application you are looking for is not listed, contact your local laboratory for assistance.
Specimen Requirements
Fasting Required Specimen Type Preferred Container/Tube Acceptable Container/Tube Specimen Volume Specimen Minimum Volume
(allows for 1 repeat)
Pediatric Minimum Volume
(no repeat)
​Yes Plasma​ Sodium-heparin Green Top Tube (GTT)​ EDTA Lavender Top Tube (LTT), Lithium-heparin Plasma Separator Tube (PST), Lithium-heparin Green Top Tube (GTT)​ 0.5 mL​ 0.3 mL​
Collection Processing

1. Patient's age is required.

2. Include family history, clinical condition (asymptomatic or acute episode), diet, and drug therapy information.

Patient Preparation: Fasting (overnight preferred, 4 hours minimum). Infants should be drawn just before next feeding (2-3 hours without total parenteral nutrition, if possible).

Collection Instructions:

1. Collect specimen and place on wet ice.

2. Centrifuge immediately or within 4 hours of collection if 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. 

Send plasma frozen. 

Specimen Stability Information
Specimen Type Temperature Time
Plasma ​Frozen ​14 days
Interference

​Reference values are for fasting patients.

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. 

Useful For
​Evaluation of 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, renal failure, and burns
Test Components
​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, phosphoserine, phosphoethanolamine, hydroxyproline, glycine, aspartic acid, ethanolamine, sarcosine, 1-methylhistidine, 3-methylhistidine, carnosine, anserine, homocitruline, alpha-aminoadipic acid, gamma-amino-n-butyric acid, beta-aminoisobutyric acid, hydroxylysine, cystathionine, and tryptophan.
Reference Range Information
See Mayo Clinic Laboratories for age specific reference ranges. 
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 of 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.
For more information visit:
Performing Laboratory Information
Performing Location Day(s) Test Performed Analytical Time Methodology/Instrumentation
Mayo Clinic Laboratories
Monday through Friday​
3 to 5 days​
Liquid Chromatography-Tandem Mass Spectrometry (LC-MS/MS)​
Reference Lab
For billing questions, see Contacts
Outreach CPTs
CPT Modifier
(if needed)
Quantity Description Comments
​82139
For most current information refer to the Marshfield Laboratory online reference manual.