Amino Acids, Quantitative, Random, Urine

Test Code

AAQIE

Alias/See Also

Epic: LAB355
Mayo: AAPD

CPT Codes
82139

Includes

TESTING ALGORITHM

Testing includes quantitation of the following amino acids: phosphoserine, phosphoethanolamine, taurine, threonine, serine, asparagine, hydroxyproline, glutamic acid, glutamine, aspartic acid, ethanolamine, sarcosine, proline, glycine, alanine, citrulline, alpha-aminoadipic acid, alpha-amino-n-butyric acid, valine, cystine, cystathionine, methionine, isoleucine, leucine, tyrosine, phenylalanine, beta-alanine, beta-aminoisobutyric acid, ornithine, lysine, 1-methylhistidine, histidine, 3-methylhistidine, carnosine, anserine, argininosuccinic acid, allo-isoleucine, homocitrulline, gamma-amino-n-butyric acid, hydroxylysine, tryptophan, and arginine.

Preferred Specimen

Specimen Type: Urine
Collection Container: Sterile container
Specimen Volume: 2 mL

Minimum Volume

1 mL

Instructions

Collect a random urine specimen.

Transport Container

Plastic vial

Specimen Stability

Specimen Type	Temperature	Time	Special Container
Urine	Frozen (preferred)	70 days
	Refrigerated	14 days

Reject Criteria (Eg, hemolysis? Lipemia? Thaw/Other?)

All specimens will be evaluated at Mayo Clinic Laboratories for test suitability.

Methodology
Liquid Chromatography Tandem Mass Spectrometry (LC-MS/MS)

Portions of this test are covered by patents held by Quest Diagnostics

Setup Schedule

Monday through Friday

Report Available

3 to 5 days

Limitations

CAUTIONS
Not all patients with homocystinuria and prolidase deficiency will be detected by this assay. See Additional Testing Requirements and Necessary Information for more information.

Reference Range

REFERENCE VALUES

Amino Acid		Age groups
		< or =12 months	13-35 months	3-6 years	7-8 years	9-17 years	> or =18 years

Phosphoserine	PSer	<1	<1	<1	<1	<1	<1
Phosphoethanolamine	PEtN	15-341	33-342	19-164	12-118	<88	<48
Taurine	Tau	37-8300	64-3255	76-3519	50-2051	57-2235	24-1531
Asparagine	Asn	25-1000	62-884	28-412	38-396	22-283	25-238
Serine	Ser	18-4483	284-1959	179-1285	153-765	105-846	97-540
Hydroxyproline	Hyp	<2536	<89	<46	<19	<22	<15
Glycine	Gly	362-18614	627-6914	412-5705	449-4492	316-4249	229-2989
Glutamine	Gln	139-2985	263-2979	152-1325	164-1125	188-1365	93-686
Aspartic Acid	Asp	<64	<56	<30	<9	<11	<10
Ethanolamine	EtN	282-3782	256-947	193-643	137-564	158-596	95-471
Histidine	His	145-3833	427-3398	230-2635	268-2147	134-1983	81-1128
Threonine	Thr	25-1217	55-763	30-554	25-456	37-418	31-278
Citrulline	Cit	<72	<57	<14	<9	<14	<12
Sarcosine	Sar	<75	<12	<9	<2	<3	<3
Beta-Alanine	bAla	<219	<92	<25	<25	<49	<52
Alanine	Ala	93-3007	101-1500	64-1299	44-814	51-696	56-518
Glutamic Acid	Glu	<243	12-128	<76	<39	<62	<34
1-Methylhistidine	1MHis	17-419	18-1629	10-1476	19-1435	12-1549	23-1339
3-Methylhistidine	3MHis	88-350	86-330	56-316	77-260	47-262	70-246
Argininosuccinic Acid	Asa	<77	<48	<37	<24	<69	<15
Carnosine	Car	27-1021	16-616	18-319	<161	<109	<35
Anserine	Ans	<277	<820	<398	<141	<369	<38
Homocitrulline	Hcit	<295	11-158	<71	<62	<33	<30
Arginine	Arg	10-560	20-395	14-240	<134	<153	<114
Alpha-aminoadipic Acid	Aad	10-275	15-324	10-135	<84	<76	<47
Gamma Amino-n-butyric Acid	GABA	<25	<13	<11	<6	<5	<5
Beta-aminoisobutyric Acid	bAib	18-3137	<980	15-1039	24-511	11-286	<301
Alpha-amino-n-butyric Acid	Abu	<63	<56	<38	<30	<31	<19
Hydroxylysine	Hyl	<150	<57	<34	<26	<31	<12
Proline	Pro	28-2029	<119	<78	<20	<28	<26
Ornithine	Orn	<265	<70	<44	<17	<18	<25
Cystathionine	Cth	<302	<56	<26	<18	<44	<30
Cystine	Cys	12-504	11-133	<130	<56	<104	10-98
Lysine	Lys	19-1988	25-743	14-307	17-276	10-240	15-271
Methionine	Met	<41	<41	<25	<23	<20	<16
Valine	Val	11-211	11-211	<139	16-91	<75	11-61
Tyrosine	Tyr	39-685	38-479	23-254	22-245	12-208	15-115
Isoleucine	Ile	<86	<78	<62	<34	<28	<22
Leucine	Leu	<200	15-167	12-100	13-73	<62	<51
Phenylalanine	Phe	14-280	34-254	20-150	21-106	11-111	13-70
Tryptophan	Trp	14-315	14-315	10-303	10-303	15-229	18-114
Allo-isoleucine	AlloIle	<29	<10	<8	<8	<8	<7

ALL RESULTS REPORTED AS NMOL/MG CREATININE.

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.

Clinical Significance

USEFUL FOR
Evaluating patients with possible inborn errors of metabolism using random urine specimens

May aid in evaluation of endocrine disorders, liver diseases, muscle diseases, neoplastic diseases, neurological disorders, nutritional disturbances, renal failure, and burns

CLINICAL INFORMATION
Amino acids are the basic structural units that comprise proteins and are found throughout the body. Many inborn errors of amino acid metabolism that affect amino acid transport or metabolism have been identified, such as phenylketonuria and tyrosinemia. 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 specfic 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.

In addition, 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. General elevations in urine amino acid levels, called aminoaciduria, can be seen in disorders with amino acid transport defects, such as lysinuric protein intolerance and Hartnup disease, as well as in conditions with renal tubular dysfunction including Lowe syndrome and Dent disease.

Performing Laboratory
Mayo Clinic Laboratories - Rochester
3050 Superior Drive NW
Rochester, MN 55901

Additional Information
Amino Acids, Quantitative, Random, Urine

Last Updated: September 5, 2023
Last Review: N. Wolford, September 5, 2023

The CPT Codes provided in this document are based on AMA guidelines and are for informational purposes only. CPT coding is the sole responsibility of the billing party. Please direct any questions regarding coding to the payor being billed. Any Profile/panel component may be ordered separately. Reflex tests are performed at an additional charge.