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Amino Acids Quantitative by LC-MS/MS, Urine
2009419
Ordering Recommendation

Screen for disorders of amino acids transport (eg, cystinuria, lysinuric protein intolerance, HHH syndrome).  Also useful to evaluate renal tubular function.

Mnemonic
URNAA QNT
Methodology
Quantitative Liquid Chromatography/Tandem Mass Spectrometry
Performed
Mon-Fri
Reported
3-7 days
New York DOH Approval Status
This test is New York DOH approved.
ARUP Consult®
Disease Topics
Specimen Required
Patient Preparation
First morning urine preferred. 
Collect
Random urine. Avoid dilute urine when possible. 
Specimen Preparation
Mix urine well. Transfer 4 mL aliquot of urine to ARUP Standard Transport Tubes and freeze immediately. (Min: 3 mL) 
Storage/Transport Temperature
CRITICAL FROZEN. Separate specimens must be submitted when multiple tests are ordered. 
Unacceptable Conditions
 
Remarks
Clinical information is needed for appropriate interpretation. Additional required information includes age, gender, diet (eg, TPN therapy), drug therapy, and family history. Biochemical Genetics Patient History Form is available on the ARUP Website or by contacting ARUP Client Services. 
Stability
Ambient: Unacceptable; Refrigerated: 24 hours; Frozen: 1 month 
Reference Interval
Effective November 18, 2019
Test Number
Components
Reference Interval
 Alpha-aminoadipic acid, Urine
AgeReference Interval
0-2 monthsLess than or equal to 700 µmol/g creatinine
3-11 monthsLess than or equal to 520 µmol/g creatinine
1-2 yearsLess than or equal to 470 µmol/g creatinine
3-5 yearsLess than or equal to 200 µmol/g creatinine
6-11 yearsLess than or equal to 125 µmol/g creatinine
12 years and olderLess than or equal to 100 µmol/g creatinine

 Alpha-amino butyric acid, Urine
Age
Reference Interval
0-2 monthsLess than or equal to 120 µmol/g creatinine
3-11 monthsLess than or equal to 80 µmol/g creatinine
1-2 yearsLess than or equal to 70 µmol/g creatinine
3-5 yearsLess than or equal to 60 µmol/g creatinine
6-11 yearsLess than or equal to 50 µmol/g creatinine
12 years and olderLess than or equal to 25 µmol/g creatinine

 Alanine, Urine
AgeReference Interval
0-2 months475-3330 µmol/g creatinine
3-11 months270-3020 µmol/g creatinine
1-2 years170-1750 µmol/g creatinine
3-5 years100-1000 µmol/g creatinine
6-11 years80-930 µmol/g creatinine
12 years and older60 -500 µmol/g creatinine

 Anserine, Urine
Age
Reference Interval
0-2 monthsLess than or equal to 60 µmol/g creatinine
3-11 monthsLess than or equal to 300 µmol/g creatinine
1-2 yearsLess than or equal to 720 µmol/g creatinine
3-5 yearsLess than or equal to 385 µmol/g creatinine
6-11 yearsLess than or equal to 480 µmol/g creatinine
12 years and olderLess than or equal to 250 µmol/g creatinine

 Arginine, Urine
AgeReference Interval
0-2 monthsLess than or equal to 470 µmol/g creatinine
3-11 monthsLess than or equal to 340 µmol/g creatinine
1-2 yearsLess than or equal to 390 µmol/g creatinine
3-5 yearsLess than or equal to 270 µmol/g creatinine
6-11 yearsLess than or equal to 160 µmol/g creatinine
12 years and olderLess than or equal to 100 µmol/g creatinine

 Argininosuccinic Acid, Urine
AgeReference Interval
0-2 monthsLess than or equal to 110 µmol/g creatinine
3-11 monthsLess than or equal to 100 µmol/g creatinine
1-2 yearsLess than or equal to 80 µmol/g creatinine
3-5 yearsLess than or equal to 65 µmol/g creatinine
6-11 yearsLess than or equal to 50 µmol/g creatinine
12 years and olderLess than or equal to 40 µmol/g creatinine

 Asparagine, Urine
AgeReference Interval
0-2 months55-1445 µmol/g creatinine
3-11 months45-910 µmol/g creatinine
1-2 years80-675 µmol/g creatinine
3-5 years50-345 µmol/g creatinine
6-11 years40-390 µmol/g creatinine
12 years and older25-180 µmol/g creatinine

 Aspartic Acid, Urine
AgeReference Interval
0-2 monthsLess than or equal to 370 µmol/g creatinine
3-11 monthsLess than or equal to 160 µmol/g creatinine
1-2 yearsLess than or equal to 65 µmol/g creatinine
3 years and olderLess than or equal to 25 µmol/g creatinine

 Beta-alanine Urine
AgeReference Interval
0-5 monthsLess than or equal to 250 µmol/g creatinine
6 months and olderLess than or equal to 125 µmol/g creatinine

 Beta-amino isobutyric acid, Urine
AgeReference Interval
0-2 monthsLess than or equal to 6780 µmol/g creatinine
3-11 monthsLess than or equal to 6000 µmol/g creatinine
1-2 yearsLess than or equal to 5500 µmol/g creatinine
3-5 yearsLess than or equal to 3490 µmol/g creatinine
6-11 yearsLess than or equal to 1720 µmol/g creatinine
12 years and olderLess than or equal to 1200 µmol/g creatinine

 Citrulline, Urine
AgeReference Interval
0-2 monthsLess than or equal to 145 µmol/g creatinine
3-11 monthsLess than or equal to 75 µmol/g creatinine
1-2 yearsLess than or equal to 40 µmol/g creatinine
3 years and olderLess than or equal to 15 µmol/g creatinine

 Cystathionine, Urine
Age
Reference Interval
0-2 monthsLess than or equal to 235 µmol/g creatinine
3-11 monthsLess than or equal to 60 µmol/g creatinine
1-2 yearsLess than or equal to 75 µmol/g creatinine
3-5 yearsLess than or equal to 35 µmol/g creatinine
6-11 yearsLess than or equal to 25 µmol/g creatinine
12 years and olderLess than or equal to 60 µmol/g creatinine

 Cystine, Urine
AgeReference Interval
0-2 monthsLess than or equal to 870 µmol/g creatinine
3-11 monthsLess than or equal to 300 µmol/g creatinine
1-2 yearsLess than or equal to 150 µmol/g creatinine
3-5 yearsLess than or equal to 125 µmol/g creatinine
6-11 yearsLess than or equal to 100 µmol/g creatinine
12 years and olderLess than or equal to 150 µmol/g creatinine

 Ethanolamine, Urine
AgeReference Interval
0-2 months390-6560 µmol/g creatinine
3-11 months320-1410 µmol/g creatinine
1-2 years270-1160 µmol/g creatinine
3-5 years245-825 µmol/g creatinine
6-11 years130-770 µmol/g creatinine
12 years and older100-510 µmol/g creatinine

 Gamma-amino butyric acid, Urine
AgeReference Interval
0-2 monthsLess than or equal to 60 µmol/g creatinine
3-5 monthsLess than or equal to 50 µmol/g creatinine
6 months and olderLess than or equal to 25 µmol/g creatinine

 Glutamic Acid, Urine
AgeReference Interval
0-2 monthsLess than or equal to 560 µmol/g creatinine
3-11 monthsLess than or equal to 360 µmol/g creatinine
1-2 yearsLess than or equal to 190 µmol/g creatinine
3-5 yearsLess than or equal to 80 µmol/g creatinine
6-11 yearsLess than or equal to 70 µmol/g creatinine
12 years and olderLess than or equal to 52 µmol/g creatinine

 Glutamine, Urine
AgeReference Interval
0-2 months380-3860 µmol/g creatinine
3-11 months310-3240 µmol/g creatinine
1-2 years340-2225 µmol/g creatinine
3-5 years300-1525 µmol/g creatinine
6-11 years165-1530 µmol/g creatinine
12 years and older100-665 µmol/g creatinine

 Glycine, Urine
AgeReference Interval
0-2 months1620-19725 µmol/g creatinine
3-11 months915-10220 µmol/g creatinine
1-2 years775-6600 µmol/g creatinine
3-5 years600-4600 µmol/g creatinine
6-11 years310-5700 µmol/g creatinine
12 years and older230 - 3510 µmol/g creatinine

 Histidine, Urine
AgeReference Interval
0-2 months325-4940 µmol/g creatinine
3-11 months290-4850 µmol/g creatinine
1-2 years340-4420 µmol/g creatinine
3-5 years315-2460 µmol/g creatinine
6-11 years160-2380 µmol/g creatinine
12 years and older80-1130 µmol/g creatinine

 Homocitrulline, Urine
AgeReference Interval
0-2 monthsLess than or equal to 675 µmol/g creatinine
3-11 monthsLess than or equal to 220 µmol/g creatinine
1-2 yearsLess than or equal to 150 µmol/g creatinine
3-5 yearsLess than or equal to 100 µmol/g creatinine
6-11 yearsLess than or equal to 70 µmol/g creatinine
12 years and olderLess than or equal to 40 µmol/g creatinine

 Hydroxylysine, Urine
AgeReference Interval
0-2 monthsLess than or equal to 510 µmol/g creatinine
3-11 monthsLess than or equal to 240 µmol/g creatinine
1-2 yearsLess than or equal to 85 µmol/g creatinine
3-5 yearsLess than or equal to 50 µmol/g creatinine
6-11 yearsLess than or equal to 40 µmol/g creatinine
12 years and olderLess than or equal to 30 µmol/g creatinine

 Hydroxyproline, Urine
AgeReference Interval
0-2 monthsLess than or equal to 6100 µmol/g creatinine
3-11 monthsLess than or equal to 1270 µmol/g creatinine
1-2 yearsLess than or equal to 100 µmol/g creatinine
3-5 yearsLess than or equal to 35 µmol/g creatinine
6-11 yearsLess than or equal to 20 µmol/g creatinine
12 years and olderLess than or equal to 30 µmol/g creatinine

 Isoleucine, Urine
AgeReference Interval
0-2 monthsLess than or equal to 360 µmol/g creatinine
3-11 monthsLess than or equal to 140 µmol/g creatinine
1-2 yearsLess than or equal to 100 µmol/g creatinine
3-5 yearsLess than or equal to 70 µmol/g creatinine
6-11 yearsLess than or equal to 60 µmol/g creatinine
12 years and olderLess than or equal to 45 µmol/g creatinine

 Leucine, Urine
AgeReference Interval
0-2 months20-420 µmol/g creatinine
3-11 months20-195 µmol/g creatinine
1-2 years20-190 µmol/g creatinine
3-5 years20-110 µmol/g creatinine
6-11 years20-100 µmol/g creatinine
12 years and olderLess than or equal to 45 µmol/g creatinine

 Lysine, Urine
AgeReference Interval
0-2 months120-2270 µmol/g creatinine
3-11 months55-1260 µmol/g creatinine
1-2 years45-930 µmol/g creatinine
3-5 years40-475 µmol/g creatinine
6-11 years25-440 µmol/g creatinine
12 years and olderLess than or equal to 355 µmol/g creatinine

 Methionine, Urine
AgeReference Interval
0-2 monthsLess than or equal to 100 µmol/g creatinine
3-11 monthsLess than or equal to 60 µmol/g creatinine
1-2 yearsLess than or equal to 50 µmol/g creatinine
3-11 yearsLess than or equal to 30 µmol/g creatinine
12 years and olderLess than or equal to 20 µmol/g creatinine

 Ornithine, Urine
AgeReference Interval
0-2 monthsLess than or equal to 475 µmol/g creatinine
3-11 monthsLess than or equal to 150 µmol/g creatinine
1-2 yearsLess than or equal to 70 µmol/g creatinine
3 years and olderLess than or equal to 30 µmol/g creatinine

 Phenylalanine, Urine
AgeReference Interval
0-2 months45-360 µmol/g creatinine
3-11 months65-370 µmol/g creatinine
1-2 years50-350 µmol/g creatinine
3-5 years35-170 µmol/g creatinine
6-11 years30-140 µmol/g creatinine
12 years and older15-85 µmol/g creatinine

 Proline, Urine
AgeReference Interval
0-2 months130-2340 µmol/g creatinine
3-11 monthsLess than or equal to 1190 µmol/g creatinine
1-2 yearsLess than or equal to 170 µmol/g creatinine
3-5 yearsLess than or equal to 60 µmol/g creatinine
6-11 yearsLess than or equal to 40 µmol/g creatinine
12 years and olderLess than or equal to 35 µmol/g creatinine

 Sarcosine, Urine
AgeReference Interval
0-2 monthsLess than or equal to 300 µmol/g creatinine
3-11 monthsLess than or equal to 75 µmol/g creatinine
1 year and olderLess than or equal to 25 µmol/g creatinine

 Serine, Urine
AgeReference Interval
0-2 months70-4125 µmol/g creatinine
3-11 months275-2730 µmol/g creatinine
1-2 years390-1890 µmol/g creatinine
3-5 years260-990 µmol/g creatinine
6-11 years130-1100 µmol/g creatinine
12 years and older90-470 µmol/g creatinine

 Taurine, Urine
AgeReference Interval
0-2 months95-9800 µmol/g creatinine
3-11 monthsLess than or equal to 7400 µmol/g creatinine
1-2 yearsLess than or equal to 9000 µmol/g creatinine
3-5 yearsLess than or equal to 4400 µmol/g creatinine
6-11 yearsLess than or equal to 3800 µmol/g creatinine
12 years and olderLess than or equal to 3200 µmol/g creatinine

 Threonine, Urine
AgeReference Interval
0-2 months125-2890 µmol/g creatinine
3-11 months50-1300 µmol/g creatinine
1-2 years85-910 µmol/g creatinine
3-5 years50-380 µmol/g creatinine
6-11 years40-470 µmol/g creatinine
12 years and older25-250 µmol/g creatinine

 Tryptophan, Urine
AgeReference Interval
0-2 months25-395 µmol/g creatinine
3-11 months45-390 µmol/g creatinine
1-2 years45-325 µmol/g creatinine
3-5 years35-150 µmol/g creatinine
6-11 years20-180 µmol/g creatinine
12 years and older15-95 µmol/g creatinine

 Tyrosine, Urine
AgeReference Interval
0-2 months50-870 µmol/g creatinine
3-11 months70-700 µmol/g creatinine
1-2 years65-560 µmol/g creatinine
3-5 years40-300 µmol/g creatinine
6-11 years40-280 µmol/g creatinine
12 years and older15-150 µmol/g creatinine

 Valine, Urine
AgeReference Interval
0-2 months40-425 µmol/g creatinine
3-11 months30-250 µmol/g creatinine
1-2 years40-280 µmol/g creatinine
3-5 years30-160 µmol/g creatinine
6-11 years20-120 µmol/g creatinine
12 years and olderLess than or equal to 55 µmol/g creatinine


Interpretive Data


Compliance Statement B: For laboratory developed tests not using a RUO kit, and for FDA approved, cleared or 510(k) exempt assays with alterations. This test was developed and its performance characteristics determined by ARUP Laboratories. The U. S. Food and Drug Administration has not approved or cleared this test; however, FDA clearance or approval is not currently required for clinical use. The results are not intended to be used as the sole means for clinical diagnosis or patient management decisions.

Note
Common indications for urine amino acid testing include clinical situations such as: 1) acute life-threatening episode, 2) failure to thrive, 3) recurrent vomiting, 4) neurological deterioration, 5) hyperammonemia, 6) lethargy, 7) metabolic acidosis, 8) testing or following therapy for a specific inborn error of metabolism (PKU, MSUD, tyrosinemia, etc.), and 9) kidney stones. Listing of clinical information is particularly important for appropriate interpretation.
Hotline History
View Hotline History
Components
Component Test Code*Component Chart NameLOINC
0020207Creatinine, Urine - per volume2161-8
0080600Alanine, Urine30068-1
0080601Arginine, Urine30062-4
0080602Asparagine, Urine28603-9
0080603Aspartic Acid, Urine30061-6
0080604Citrulline, Urine30161-4
0080605Cystine, Urine30065-7
0080606Glutamic Acid, Urine30059-0
0080607Glutamine, Urine30056-6
0080609Glycine, Urine30066-5
0080610Histidine, Urine30047-5
0080612Hydroxyproline, Urine28601-3
0080613Isoleucine, Urine30052-5
0080614Leucine, Urine30053-3
0080615Lysine, Urine30048-3
0080616Methionine, Urine30063-2
0080617Ornithine, Urine30049-1
0080618Phenylalanine, Urine30055-8
0080619Proline, Urine30067-3
0080620Serine, Urine30058-2
0080621Taurine, Urine28595-7
0080622Threonine, Urine30057-4
0080623Tyrosine, Urine30054-1
0080624Valine, Urine30064-0
0080952Amino Acids, Urine Interpretation49248-8
2009422Alpha-amino butyric acid, Urine28590-8
2009423Alpha-aminoadipic acid, Urine28598-1
2009424Argininosuccinic Acid, Urine32229-7
2009425Beta-amino isobutyric acid, Urine28602-1
2009426Beta-alanine Urine28588-2
2009428Ethanolamine, Urine28605-4
2009429Gamma-amino butyric acid, Urine28593-2
2009432Sarcosine, Urine28610-4
2009433Tryptophan, Urine28608-8
2009434Anserine, Urine28596-5
2009436Cystathionine, Urine28599-9
2009437Homocitrulline, Urine32248-7
2009438Hydroxylysine, Urine30050-9
* Component test codes cannot be used to order tests. The information provided here is not sufficient for interface builds; for a complete test mix, please click the sidebar link to access the Interface Map.
Aliases
  • nothing listed
  • Quantitative Plasma & Urinary Amino Acids