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22224 Amino Acids, Quantitative, Plasma (AAQP)

Amino Acids, Quantitative, Plasma (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)​
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. Note: Thrombin-activated tubes should not be used for collection.​

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 Report Available Methodology/Instrumentation
Mayo Clinic Laboratories
Monday through Friday​
3 to 5 days​
Liquid Chromatography-Tandem Mass Spectrometry (LC-MS/MS)​
Reference Lab
Test Information

​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 particular 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 disability 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 accumulate 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 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
COM ​Amino Acids Panel Qnt, Plasma (AAQP)
​Cerner​Amino Acids, Quantitative 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)​
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. Note: Thrombin-activated tubes should not be used for collection.​

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 Report Available 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.