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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
Test Number
Components
Reference Interval
 Alpha-aminoadipic acid, Urine
0-2 months
3-8 months
9 months-2 years
3-12 years
13 years and older
µmol/g creatinine
µmol/g creatinine
µmol/g creatinine
µmol/g creatinine
µmol/g creatinine
6 - 52234 - 35334 - 42515 - 2717 - 57

 Alpha-amino butyric acid, Urine
0-2 months
3-8 months
9 months-2 years
3-12 years
13 years and older
µmol/g creatinine
µmol/g creatinine
µmol/g creatinine
µmol/g creatinine
µmol/g creatinine
0 - 840 - 701 - 960 - 510 - 20

 Alanine, Urine
0-2 months
3-8 months
9 months-2 years
3-12 years
13 years and older
µmol/g creatinine
µmol/g creatinine
µmol/g creatinine
µmol/g creatinine
µmol/g creatinine
658 - 3424601 - 2918224 - 2210176 - 125578 - 587

 Anserine, Urine
0-2 months
3-8 months
9 months-2 years
3-12 years
13 years and older
µmol/g creatinine
µmol/g creatinine
µmol/g creatinine
µmol/g creatinine
µmol/g creatinine
0 - 1090 - 2180 - 6350 - 6000 - 67

 Arginine, Urine
0-2 months
3-8 months
9 months-2 years
3-12 years
13 years and older
µmol/g creatinine
µmol/g creatinine
µmol/g creatinine
µmol/g creatinine
µmol/g creatinine
0 - 3130 - 3070 - 4240 - 1505 - 34

 Argininosuccinic Acid, Urine
0-2 months
3-8 months
9 months-2 years
3-12 years
13 years and older
µmol/g creatinine
µmol/g creatinine
µmol/g creatinine
µmol/g creatinine
µmol/g creatinine
0 - 1020 - 1390 - 1290 - 640 - 68

 Asparagine, Urine
0-2 months
3-8 months
9 months-2 years
3-12 years
13 years and older
µmol/g creatinine
µmol/g creatinine
µmol/g creatinine
µmol/g creatinine
µmol/g creatinine
0 - 12600 - 8540 - 75849 - 46622 - 170

 Aspartic Acid, Urine
0-2 months
3-8 months
9 months-2 years
3-12 years
13 years and older
µmol/g creatinine
µmol/g creatinine
µmol/g creatinine
µmol/g creatinine
µmol/g creatinine
0 - 8660 - 2992 - 1380 - 600 - 26

 Beta-alanine Urine
0-2 months
3-8 months
9 months-2 years
3-12 years
13 years and older
µmol/g creatinine
µmol/g creatinine
µmol/g creatinine
µmol/g creatinine
µmol/g creatinine
28 - 7050 - 7310 - 2180 - 1950 - 130

 Beta-amino isobutyric acid, Urine
0-2 months
3-8 months
9 months-2 years
3-12 years
13 years and older
µmol/g creatinine
µmol/g creatinine
µmol/g creatinine
µmol/g creatinine
µmol/g creatinine
0 - 891891 - 714064 - 910018 - 49232 - 922

 Citrulline, Urine
0-2 months
3-8 months
9 months-2 years
3-12 years
13 years and older
µmol/g creatinine
µmol/g creatinine
µmol/g creatinine
µmol/g creatinine
µmol/g creatinine
0 - 1650 - 820 - 760 - 220 - 12

 Cystathionine, Urine
0-2 months
3-8 months
9 months-2 years
3-12 years
13 years and older
µmol/g creatinine
µmol/g creatinine
µmol/g creatinine
µmol/g creatinine
µmol/g creatinine
0 - 2270 - 1710 - 1310 - 660 - 34

 Cystine, Urine
0-2 months
3-8 months
9 months-2 years
3-12 years
13 years and older
µmol/g creatinine
µmol/g creatinine
µmol/g creatinine
µmol/g creatinine
µmol/g creatinine
14 - 57328 - 46134 - 18626 - 9812 - 81

 Ethanolamine, Urine
0-2 months
3-8 months
9 months-2 years
3-12 years
13 years and older
µmol/g creatinine
µmol/g creatinine
µmol/g creatinine
µmol/g creatinine
µmol/g creatinine
0 - 23620 - 2251156 - 146260 - 714123 - 610

 Gamma-amino butyric acid, Urine
0-2 months
3-8 months
9 months-2 years
3-12 years
13 years and older
µmol/g creatinine
µmol/g creatinine
µmol/g creatinine
µmol/g creatinine
µmol/g creatinine
0 - 310 - 160 - 160 - 60 - 4

 Glutamic Acid, Urine
0-2 months
3-8 months
9 months-2 years
3-12 years
13 years and older
µmol/g creatinine
µmol/g creatinine
µmol/g creatinine
µmol/g creatinine
µmol/g creatinine
17 - 101265 - 115518 - 1869 - 914 - 38

 Glutamine, Urine
0-2 months
3-8 months
9 months-2 years
3-12 years
13 years and older
µmol/g creatinine
µmol/g creatinine
µmol/g creatinine
µmol/g creatinine
µmol/g creatinine
117 - 40860 - 2916480 - 3070300 - 1896110 - 609

 Glycine, Urine
0-2 months
3-8 months
9 months-2 years
3-12 years
13 years and older
µmol/g creatinine
µmol/g creatinine
µmol/g creatinine
µmol/g creatinine
µmol/g creatinine
1970 - 169401613 - 14465837 - 6625595 - 5432296 - 4419

 Histidine, Urine
0-2 months
3-8 months
9 months-2 years
3-12 years
13 years and older
µmol/g creatinine
µmol/g creatinine
µmol/g creatinine
µmol/g creatinine
µmol/g creatinine
405 - 4186421 - 3393435 - 3986259 - 2070100 - 1004

 Homocitrulline, Urine
0-2 months
3-8 months
9 months-2 years
3-12 years
13 years and older
µmol/g creatinine
µmol/g creatinine
µmol/g creatinine
µmol/g creatinine
µmol/g creatinine
0 - 3080 - 1110 - 1410 - 1403 - 52

 Hydroxylysine, Urine
0-2 months
3-8 months
9 months-2 years
3-12 years
13 years and older
µmol/g creatinine
µmol/g creatinine
µmol/g creatinine
µmol/g creatinine
µmol/g creatinine
0 - 3830 - 2050 - 1430 - 700 - 56

 Hydroxyproline, Urine
0-2 months
3-8 months
9 months-2 years
3-12 years
13 years and older
µmol/g creatinine
µmol/g creatinine
µmol/g creatinine
µmol/g creatinine
µmol/g creatinine
83 - 54300 - 25610 - 11910 - 540 - 15

 Isoleucine, Urine
0-2 months
3-8 months
9 months-2 years
3-12 years
13 years and older
µmol/g creatinine
µmol/g creatinine
µmol/g creatinine
µmol/g creatinine
µmol/g creatinine
17 - 31222 - 15914 - 1078 - 8710 - 32

 Leucine, Urine
0-2 months
3-8 months
9 months-2 years
3-12 years
13 years and older
µmol/g creatinine
µmol/g creatinine
µmol/g creatinine
µmol/g creatinine
µmol/g creatinine
9 - 33637 - 25322 - 21520 - 1297 - 50

 Lysine, Urine
0-2 months
3-8 months
9 months-2 years
3-12 years
13 years and older
µmol/g creatinine
µmol/g creatinine
µmol/g creatinine
µmol/g creatinine
µmol/g creatinine
58 - 224652 - 171649 - 95331 - 39416 - 271

 Methionine, Urine
0-2 months
3-8 months
9 months-2 years
3-12 years
13 years and older
µmol/g creatinine
µmol/g creatinine
µmol/g creatinine
µmol/g creatinine
µmol/g creatinine
0 - 840 - 690 - 592 - 341 - 22

 Ornithine, Urine
0-2 months
3-8 months
9 months-2 years
3-12 years
13 years and older
µmol/g creatinine
µmol/g creatinine
µmol/g creatinine
µmol/g creatinine
µmol/g creatinine
0 - 2162 - 1511 - 1002 - 750 - 24

 Phenylalanine, Urine
0-2 months
3-8 months
9 months-2 years
3-12 years
13 years and older
µmol/g creatinine
µmol/g creatinine
µmol/g creatinine
µmol/g creatinine
µmol/g creatinine
30 - 35661 - 35753 - 36427 - 18712 - 92

 Proline, Urine
0-2 months
3-8 months
9 months-2 years
3-12 years
13 years and older
µmol/g creatinine
µmol/g creatinine
µmol/g creatinine
µmol/g creatinine
µmol/g creatinine
50 - 248412 - 123710 - 6626 - 1173 - 23

 Sarcosine, Urine
0-2 months
3-8 months
9 months-2 years
3-12 years
13 years and older
µmol/g creatinine
µmol/g creatinine
µmol/g creatinine
µmol/g creatinine
µmol/g creatinine
11 - 4311 - 1381 - 541 - 100 - 4

 Serine, Urine
0-2 months
3-8 months
9 months-2 years
3-12 years
13 years and older
µmol/g creatinine
µmol/g creatinine
µmol/g creatinine
µmol/g creatinine
µmol/g creatinine
51 - 2910173 - 3399364 - 2732100 - 94764 - 501

 Taurine, Urine
0-2 months
3-8 months
9 months-2 years
3-12 years
13 years and older
µmol/g creatinine
µmol/g creatinine
µmol/g creatinine
µmol/g creatinine
µmol/g creatinine
142 - 9594100 - 741062 - 557260 - 219819 - 2859

 Threonine, Urine
0-2 months
3-8 months
9 months-2 years
3-12 years
13 years and older
µmol/g creatinine
µmol/g creatinine
µmol/g creatinine
µmol/g creatinine
µmol/g creatinine
54 - 233467 - 1380118 - 131840 - 58533 - 209

 Tryptophan, Urine
0-2 months
3-8 months
9 months-2 years
3-12 years
13 years and older
µmol/g creatinine
µmol/g creatinine
µmol/g creatinine
µmol/g creatinine
µmol/g creatinine
40 - 40276 - 38860 - 38636 - 18313 - 94

 Tyrosine, Urine
0-2 months
3-8 months
9 months-2 years
3-12 years
13 years and older
µmol/g creatinine
µmol/g creatinine
µmol/g creatinine
µmol/g creatinine
µmol/g creatinine
70 - 857126 - 77576 - 65959 - 41018 - 170

 Valine, Urine
0-2 months
3-8 months
9 months-2 years
3-12 years
13 years and older
µmol/g creatinine
µmol/g creatinine
µmol/g creatinine
µmol/g creatinine
µmol/g creatinine
41 - 42860 - 28030 - 33826 - 19110 - 73


Interpretive Data


Compliance Statement B: 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.
CPT Code(s)
82139
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 Interpretation49250-4
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