Amino Acids Quantitative by LC-MS/MS, Urine
2009419
 
Ordering Recommendation
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.
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
α-​Aminoadipic acid
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 -​ 522 34 -​ 353 34 -​ 425 15 -​ 271 7 -​ 57
α-​Amino-​n-​butyric acid
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 -​ 84 0 -​ 70 1 -​ 96 0 -​ 51 0 -​ 20
Alanine
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 -​ 3424 601 -​ 2918 224 -​ 2210 176 -​ 1255 78 -​ 587
Anserine
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 -​ 109 0 -​ 218 0 -​ 635 0 -​ 600 0 -​ 67
Arginine
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 -​ 313 0 -​ 307 0 -​ 424 0 -​ 150 5 -​ 34
Argininosuccinic acid
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 -​ 102 0 -​ 139 0 -​ 129 0 -​ 64 0 -​ 68
Asparagine
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 -​ 1260 0 -​ 854 0 -​ 758 49 -​ 466 22 -​ 170
Aspartic acid
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 -​ 866 0 -​ 299 2 -​ 138 0 -​ 60 0 -​ 26
β-​Alanine
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 -​ 705 0 -​ 731 0 -​ 218 0 -​ 195 0 -​ 130
β-​Aminoisobutyric acid
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 -​ 8918 91 -​ 7140 64 -​ 9100 18 -​ 4923 2 -​ 922
Citrulline
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 -​ 165 0 -​ 82 0 -​ 76 0 -​ 22 0 -​ 12
Cystathionine
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 -​ 227 0 -​ 171 0 -​ 131 0 -​ 66 0 -​ 34
Cystine
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 -​ 573 28 -​ 461 34 -​ 186 26 -​ 98 12 -​ 81
Ethanolamine
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 -​ 2362 0 -​ 2251 156 -​ 1462 60 -​ 714 123 -​ 610
γ-​Amino-​n-​butyric acid
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 -​ 31 0 -​ 16 0 -​ 16 0 -​ 6 0 -​ 4
Glutamic acid
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 -​ 1012 65 -​ 1155 18 -​ 186 9 -​ 91 4 -​ 38
Glutamine
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 -​ 4086 0 -​ 2916 480 -​ 3070 300 -​ 1896 110 -​ 609
Glycine
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 -​ 16940 1613 -​ 14465 837 -​ 6625 595 -​ 5432 296 -​ 4419
Histidine
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 -​ 4186 421 -​ 3393 435 -​ 3986 259 -​ 2070 100 -​ 1004
Homocitrulline
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 -​ 308 0 -​ 111 0 -​ 141 0 -​ 140 3 -​ 52
Hydroxylysine
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 -​ 383 0 -​ 205 0 -​ 143 0 -​ 70 0 -​ 56
Hydroxyproline
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 -​ 5430 0 -​ 2561 0 -​ 1191 0 -​ 54 0 -​ 15
Isoleucine
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 -​ 312 22 -​ 159 14 -​ 107 8 -​ 87 10 -​ 32
Leucine
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 -​ 336 37 -​ 253 22 -​ 215 20 -​ 129 7 -​ 50
Lysine
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 -​ 2246 52 -​ 1716 49 -​ 953 31 -​ 394 16 -​ 271
Methionine
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 -​ 84 0 -​ 69 0 -​ 59 2 -​ 34 1 -​ 22
Ornithine
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 -​ 216 2 -​ 151 1 -​ 100 2 -​ 75 0 -​ 24
Phenylalaline
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 -​ 356 61 -​ 357 53 -​ 364 27 -​ 187 12 -​ 92
Proline
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 -​ 2484 12 -​ 1237 10 -​ 662 6 -​ 117 3 -​ 23
Sarcosine
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 -​ 431 1 -​ 138 1 -​ 54 1 -​ 10 0 -​ 4
Serine
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 -​ 2910 173 -​ 3399 364 -​ 2732 100 -​ 947 64 -​ 501
Taurine
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 -​ 9594 100 -​ 7410 62 -​ 5572 60 -​ 2198 19 -​ 2859
Threonine
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 -​ 2334 67 -​ 1380 118 -​ 1318 40 -​ 585 33 -​ 209
Tryptophan
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 -​ 402 76 -​ 388 60 -​ 386 36 -​ 183 13 -​ 94
Tyrosine
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 -​ 857 126 -​ 775 76 -​ 659 59 -​ 410 18 -​ 170
Valine
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 -​ 428 60 -​ 280 30 -​ 338 26 -​ 191 10 -​ 73
Interpretive Data


See Compliance Statement B: www.aruplab.com/CS
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 Name
0020207Creatinine, Urine - per volume
0080600Alanine, Urine
0080601Arginine, Urine
0080602Asparagine, Urine
0080603Aspartic Acid, Urine
0080604Citrulline, Urine
0080605Cystine, Urine
0080606Glutamic Acid, Urine
0080607Glutamine, Urine
0080609Glycine, Urine
0080610Histidine, Urine
0080612Hydroxyproline, Urine
0080613Isoleucine, Urine
0080614Leucine, Urine
0080615Lysine, Urine
0080616Methionine, Urine
0080617Ornithine, Urine
0080618Phenylalanine, Urine
0080619Proline, Urine
0080620Serine, Urine
0080621Taurine, Urine
0080622Threonine, Urine
0080623Tyrosine, Urine
0080624Valine, Urine
0080952Amino Acids, Urine Interpretation
2009422Alpha-amino butyric acid, Urine
2009423Alpha-aminoadipic acid, Urine
2009424Argininosuccinic Acid, Urine
2009425Beta-amino isobutyric acid, Urine
2009426Beta-alanine Urine
2009428Ethanolamine, Urine
2009429Gamma-amino butyric acid, Urine
2009432Sarcosine, Urine
2009433Tryptophan, Urine
2009434Anserine, Urine
2009436Cystathionine, Urine
2009437Homocitrulline, Urine
2009438Hydroxylysine, Urine
* Component test codes cannot be used to order tests. The information provided here is not sufficient for interface builds; for a complete test mix, contact interface support at interface.support@aruplab.com.
Cross References
  • nothing listed
  • Quantitative Plasma & Urinary Amino Acids