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22224 Amino Acids, Quantitative Panel (AAQP)

Amino Acids, Quantitative Panel (AAQP)
Test Code: AAIEPSO
Synonyms/Keywords
​Ref Lab Code: 9265, 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 AciduriaCarbamoyl 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
​Evaluating patients with possible inborn errors of metabolism
 
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
​Submit sample in a plastic vial.
1. Fasting for 12-14 hrs (4 hours or more in infants).
2. Spin down promptly.
Additional Information: 
1. Patient's age is required.
2. Include family history, clinical condition (asymptomatic or acute episode), diet, and drug therapy information.
Specimen Stability Information
Specimen Type Temperature Time
​Plasma ​Frozen ​14 days
Rejection Criteria
Thrombin-activated tube
Performing Laboratory Information
Performing Location Day(s) Test Performed Analytical Time Methodology/Instrumentation
Mayo Medical Laboratories​
 Monday through Friday​
3 days (not reported on Saturday or Sunday)​
 Quantitative Analysis by Liquid Chromatography-Tandem Mass Spectrometry (LC-MS/MS)​
Reference Lab
Mayo Clinic Laboratories
Reference Range Information
Interpretive report
Interpretation
​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 at Mayo or elsewhere, 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
​Ref Lab Code: 9265, 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 AciduriaCarbamoyl 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
​Submit sample in a plastic vial.
1. Fasting for 12-14 hrs (4 hours or more in infants).
2. Spin down promptly.
Additional Information: 
1. Patient's age is required.
2. Include family history, clinical condition (asymptomatic or acute episode), diet, and drug therapy information.
Specimen Stability Information
Specimen Type Temperature Time
​Plasma ​Frozen ​14 days
Rejection Criteria
Thrombin-activated tube
Useful For
​Evaluating patients with possible inborn errors of metabolism
 
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
Interpretive report
Interpretation
​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 at Mayo or elsewhere, 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 Medical Laboratories​
 Monday through Friday​
3 days (not reported on Saturday or Sunday)​
 Quantitative Analysis by Liquid Chromatography-Tandem Mass Spectrometry (LC-MS/MS)​
Reference Lab
Mayo Clinic Laboratories
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.