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.
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
Age Reference Interval
0-2 months Less than or equal to 700 µmol/g creatinine
3-11 months Less than or equal to 520 µmol/g creatinine
1-2 years Less than or equal to 470 µmol/g creatinine
3-5 years Less than or equal to 200 µmol/g creatinine
6-11 years Less than or equal to 125 µmol/g creatinine
12 years and older Less than or equal to 100 µmol/g creatinine

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

  Alanine, Urine
Age Reference Interval
0-2 months 475-3330 µmol/g creatinine
3-11 months 270-3020 µmol/g creatinine
1-2 years 170-1750 µmol/g creatinine
3-5 years 100-1000 µmol/g creatinine
6-11 years 80-930 µmol/g creatinine
12 years and older 60 -500 µmol/g creatinine

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

  Arginine, Urine
Age Reference Interval
0-2 months Less than or equal to 470 µmol/g creatinine
3-11 months Less than or equal to 340 µmol/g creatinine
1-2 years Less than or equal to 390 µmol/g creatinine
3-5 years Less than or equal to 270 µmol/g creatinine
6-11 years Less than or equal to 160 µmol/g creatinine
12 years and older Less than or equal to 100 µmol/g creatinine

  Argininosuccinic Acid, Urine
Age Reference Interval
0-2 months Less than or equal to 110 µmol/g creatinine
3-11 months Less than or equal to 100 µmol/g creatinine
1-2 years Less than or equal to 80 µmol/g creatinine
3-5 years Less than or equal to 65 µmol/g creatinine
6-11 years Less than or equal to 50 µmol/g creatinine
12 years and older Less than or equal to 40 µmol/g creatinine

  Asparagine, Urine
Age Reference Interval
0-2 months 55-1445 µmol/g creatinine
3-11 months 45-910 µmol/g creatinine
1-2 years 80-675 µmol/g creatinine
3-5 years 50-345 µmol/g creatinine
6-11 years 40-390 µmol/g creatinine
12 years and older 25-180 µmol/g creatinine

  Aspartic Acid, Urine
Age Reference Interval
0-2 months Less than or equal to 370 µmol/g creatinine
3-11 months Less than or equal to 160 µmol/g creatinine
1-2 years Less than or equal to 65 µmol/g creatinine
3 years and older Less than or equal to 25 µmol/g creatinine

  Beta-alanine Urine
Age Reference Interval
0-5 months Less than or equal to 250 µmol/g creatinine
6 months and older Less than or equal to 125 µmol/g creatinine

  Beta-amino isobutyric acid, Urine
Age Reference Interval
0-2 months Less than or equal to 6780 µmol/g creatinine
3-11 months Less than or equal to 6000 µmol/g creatinine
1-2 years Less than or equal to 5500 µmol/g creatinine
3-5 years Less than or equal to 3490 µmol/g creatinine
6-11 years Less than or equal to 1720 µmol/g creatinine
12 years and older Less than or equal to 1200 µmol/g creatinine

  Citrulline, Urine
Age Reference Interval
0-2 months Less than or equal to 145 µmol/g creatinine
3-11 months Less than or equal to 75 µmol/g creatinine
1-2 years Less than or equal to 40 µmol/g creatinine
3 years and older Less than or equal to 15 µmol/g creatinine

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

  Cystine, Urine
Age Reference Interval
0-2 months Less than or equal to 870 µmol/g creatinine
3-11 months Less than or equal to 300 µmol/g creatinine
1-2 years Less than or equal to 150 µmol/g creatinine
3-5 years Less than or equal to 125 µmol/g creatinine
6-11 years Less than or equal to 100 µmol/g creatinine
12 years and older Less than or equal to 150 µmol/g creatinine

  Ethanolamine, Urine
Age Reference Interval
0-2 months 390-6560 µmol/g creatinine
3-11 months 320-1410 µmol/g creatinine
1-2 years 270-1160 µmol/g creatinine
3-5 years 245-825 µmol/g creatinine
6-11 years 130-770 µmol/g creatinine
12 years and older 100-510 µmol/g creatinine

  Gamma-amino butyric acid, Urine
Age Reference Interval
0-2 months Less than or equal to 60 µmol/g creatinine
3-5 months Less than or equal to 50 µmol/g creatinine
6 months and older Less than or equal to 25 µmol/g creatinine

  Glutamic Acid, Urine
Age Reference Interval
0-2 months Less than or equal to 560 µmol/g creatinine
3-11 months Less than or equal to 360 µmol/g creatinine
1-2 years Less than or equal to 190 µmol/g creatinine
3-5 years Less than or equal to 80 µmol/g creatinine
6-11 years Less than or equal to 70 µmol/g creatinine
12 years and older Less than or equal to 52 µmol/g creatinine

  Glutamine, Urine
Age Reference Interval
0-2 months 380-3860 µmol/g creatinine
3-11 months 310-3240 µmol/g creatinine
1-2 years 340-2225 µmol/g creatinine
3-5 years 300-1525 µmol/g creatinine
6-11 years 165-1530 µmol/g creatinine
12 years and older 100-665 µmol/g creatinine

  Glycine, Urine
Age Reference Interval
0-2 months 1620-19725 µmol/g creatinine
3-11 months 915-10220 µmol/g creatinine
1-2 years 775-6600 µmol/g creatinine
3-5 years 600-4600 µmol/g creatinine
6-11 years 310-5700 µmol/g creatinine
12 years and older 230 - 3510 µmol/g creatinine

  Histidine, Urine
Age Reference Interval
0-2 months 325-4940 µmol/g creatinine
3-11 months 290-4850 µmol/g creatinine
1-2 years 340-4420 µmol/g creatinine
3-5 years 315-2460 µmol/g creatinine
6-11 years 160-2380 µmol/g creatinine
12 years and older 80-1130 µmol/g creatinine

  Homocitrulline, Urine
Age Reference Interval
0-2 months Less than or equal to 675 µmol/g creatinine
3-11 months Less than or equal to 220 µmol/g creatinine
1-2 years Less than or equal to 150 µmol/g creatinine
3-5 years Less than or equal to 100 µmol/g creatinine
6-11 years Less than or equal to 70 µmol/g creatinine
12 years and older Less than or equal to 40 µmol/g creatinine

  Hydroxylysine, Urine
Age Reference Interval
0-2 months Less than or equal to 510 µmol/g creatinine
3-11 months Less than or equal to 240 µmol/g creatinine
1-2 years Less than or equal to 85 µmol/g creatinine
3-5 years Less than or equal to 50 µmol/g creatinine
6-11 years Less than or equal to 40 µmol/g creatinine
12 years and older Less than or equal to 30 µmol/g creatinine

  Hydroxyproline, Urine
Age Reference Interval
0-2 months Less than or equal to 6100 µmol/g creatinine
3-11 months Less than or equal to 1270 µmol/g creatinine
1-2 years Less than or equal to 100 µmol/g creatinine
3-5 years Less than or equal to 35 µmol/g creatinine
6-11 years Less than or equal to 20 µmol/g creatinine
12 years and older Less than or equal to 30 µmol/g creatinine

  Isoleucine, Urine
Age Reference Interval
0-2 months Less than or equal to 360 µmol/g creatinine
3-11 months Less than or equal to 140 µmol/g creatinine
1-2 years Less than or equal to 100 µmol/g creatinine
3-5 years Less than or equal to 70 µmol/g creatinine
6-11 years Less than or equal to 60 µmol/g creatinine
12 years and older Less than or equal to 45 µmol/g creatinine

  Leucine, Urine
Age Reference Interval
0-2 months 20-420 µmol/g creatinine
3-11 months 20-195 µmol/g creatinine
1-2 years 20-190 µmol/g creatinine
3-5 years 20-110 µmol/g creatinine
6-11 years 20-100 µmol/g creatinine
12 years and older Less than or equal to 45 µmol/g creatinine

  Lysine, Urine
Age Reference Interval
0-2 months 120-2270 µmol/g creatinine
3-11 months 55-1260 µmol/g creatinine
1-2 years 45-930 µmol/g creatinine
3-5 years 40-475 µmol/g creatinine
6-11 years 25-440 µmol/g creatinine
12 years and older Less than or equal to 355 µmol/g creatinine

  Methionine, Urine
Age Reference Interval
0-2 months Less than or equal to 100 µmol/g creatinine
3-11 months Less than or equal to 60 µmol/g creatinine
1-2 years Less than or equal to 50 µmol/g creatinine
3-11 years Less than or equal to 30 µmol/g creatinine
12 years and older Less than or equal to 20 µmol/g creatinine

  Ornithine, Urine
Age Reference Interval
0-2 months Less than or equal to 475 µmol/g creatinine
3-11 months Less than or equal to 150 µmol/g creatinine
1-2 years Less than or equal to 70 µmol/g creatinine
3 years and older Less than or equal to 30 µmol/g creatinine

  Phenylalanine, Urine
Age Reference Interval
0-2 months 45-360 µmol/g creatinine
3-11 months 65-370 µmol/g creatinine
1-2 years 50-350 µmol/g creatinine
3-5 years 35-170 µmol/g creatinine
6-11 years 30-140 µmol/g creatinine
12 years and older 15-85 µmol/g creatinine

  Proline, Urine
Age Reference Interval
0-2 months 130-2340 µmol/g creatinine
3-11 months Less than or equal to 1190 µmol/g creatinine
1-2 years Less than or equal to 170 µmol/g creatinine
3-5 years Less than or equal to 60 µmol/g creatinine
6-11 years Less than or equal to 40 µmol/g creatinine
12 years and older Less than or equal to 35 µmol/g creatinine

  Sarcosine, Urine
Age Reference Interval
0-2 months Less than or equal to 300 µmol/g creatinine
3-11 months Less than or equal to 75 µmol/g creatinine
1 year and older Less than or equal to 25 µmol/g creatinine

  Serine, Urine
Age Reference Interval
0-2 months 70-4125 µmol/g creatinine
3-11 months 275-2730 µmol/g creatinine
1-2 years 390-1890 µmol/g creatinine
3-5 years 260-990 µmol/g creatinine
6-11 years 130-1100 µmol/g creatinine
12 years and older 90-470 µmol/g creatinine

  Taurine, Urine
Age Reference Interval
0-2 months 95-9800 µmol/g creatinine
3-11 months Less than or equal to 7400 µmol/g creatinine
1-2 years Less than or equal to 9000 µmol/g creatinine
3-5 years Less than or equal to 4400 µmol/g creatinine
6-11 years Less than or equal to 3800 µmol/g creatinine
12 years and older Less than or equal to 3200 µmol/g creatinine

  Threonine, Urine
Age Reference Interval
0-2 months 125-2890 µmol/g creatinine
3-11 months 50-1300 µmol/g creatinine
1-2 years 85-910 µmol/g creatinine
3-5 years 50-380 µmol/g creatinine
6-11 years 40-470 µmol/g creatinine
12 years and older 25-250 µmol/g creatinine

  Tryptophan, Urine
Age Reference Interval
0-2 months 25-395 µmol/g creatinine
3-11 months 45-390 µmol/g creatinine
1-2 years 45-325 µmol/g creatinine
3-5 years 35-150 µmol/g creatinine
6-11 years 20-180 µmol/g creatinine
12 years and older 15-95 µmol/g creatinine

  Tyrosine, Urine
Age Reference Interval
0-2 months 50-870 µmol/g creatinine
3-11 months 70-700 µmol/g creatinine
1-2 years 65-560 µmol/g creatinine
3-5 years 40-300 µmol/g creatinine
6-11 years 40-280 µmol/g creatinine
12 years and older 15-150 µmol/g creatinine

  Valine, Urine
Age Reference Interval
0-2 months 40-425 µmol/g creatinine
3-11 months 30-250 µmol/g creatinine
1-2 years 40-280 µmol/g creatinine
3-5 years 30-160 µmol/g creatinine
6-11 years 20-120 µmol/g creatinine
12 years and older Less 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
N/A
CPT Codes

82139

Components
Component Test Code* Component Chart Name LOINC
0080600 Alanine, Urine 30068-1
0080601 Arginine, Urine 30062-4
0080602 Asparagine, Urine 28603-9
0080603 Aspartic Acid, Urine 30061-6
0080604 Citrulline, Urine 30161-4
0080605 Cystine, Urine 30065-7
0080606 Glutamic Acid, Urine 30059-0
0080607 Glutamine, Urine 30056-6
0080609 Glycine, Urine 30066-5
0080610 Histidine, Urine 30047-5
0080612 Hydroxyproline, Urine 28601-3
0080613 Isoleucine, Urine 30052-5
0080614 Leucine, Urine 30053-3
0080615 Lysine, Urine 30048-3
0080616 Methionine, Urine 30063-2
0080617 Ornithine, Urine 30049-1
0080618 Phenylalanine, Urine 30055-8
0080619 Proline, Urine 30067-3
0080620 Serine, Urine 30058-2
0080621 Taurine, Urine 28595-7
0080622 Threonine, Urine 30057-4
0080623 Tyrosine, Urine 30054-1
0080624 Valine, Urine 30064-0
0080952 Amino Acids, Urine Interpretation 49248-8
2009422 Alpha-amino butyric acid, Urine 28590-8
2009423 Alpha-aminoadipic acid, Urine 28598-1
2009424 Argininosuccinic Acid, Urine 32229-7
2009425 Beta-amino isobutyric acid, Urine 28602-1
2009426 Beta-alanine Urine 28588-2
2009428 Ethanolamine, Urine 28605-4
2009429 Gamma-amino butyric acid, Urine 28593-2
2009432 Sarcosine, Urine 28610-4
2009433 Tryptophan, Urine 28608-8
2009434 Anserine, Urine 28596-5
2009436 Cystathionine, Urine 28599-9
2009437 Homocitrulline, Urine 32248-7
2009438 Hydroxylysine, Urine 30050-9
3002334 Creatinine, Urine 2161-8
* 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
Amino Acids Quantitative by LC-MS/MS, Urine