Amino Acid Profile, Quantitative, Urine

CPT: 82139
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Test Details

Use

Diagnosis and monitoring of inherited aminoacidurias, organic acidurias, and urea cycle defects. May be used as a follow-up confirmatory test to some abnormal newborn screen results. In most, but not all, cases where an amino acid is elevated in blood, it will also be elevated in urine. Some disorders, primarily those involving defective renal transport, will only manifest elevated amino acids in urine. In general, urinary amino acid levels are more variable than plasma levels. For this reason, screening for aminoacidopathies in urine alone is discouraged unless a disorder is suspected that predominantly manifests abnormalities in urine (eg, cystinuria, renal Fanconi syndrome).

Limitations

False-negative results can occur, especially when a patient is affected with a mild or intermittent variant of a disorder or when a clinical condition or dietary treatment causes secondary changes to the profile. Results of amino acid profiling should be interpreted in the context of clinical presentation as well as other laboratory tests.

This test was developed, and its performance characteristics determined, by LabCorp. It has not been cleared or approved by the US Food and Drug Administration (FDA).

Methodology

Liquid chromatography/tandem mass spectrometry (LC/MS-MS)

Reference Interval

See Urine Amino Acid Reference Intervals in the Test Resources pages online.

Specimen Requirements

Specimen

Urine, frozen

Volume

5 mL

Minimum Volume

2 mL

Container

Plastic urine container without preservative

Storage Instructions

Freeze.

Stability Requirements

Temperature

Period

Room temperature

Unstable

Refrigerated

Unstable

Frozen

14 days

Causes for Rejection

Room temperature or thawed specimens

Clinical Information

References

Bremer HJ, Duran M, Kamerling JP, et al. Disturbances of Amino Acid Metabolism: Clinical Chemistry and Diagnosis. Baltimore, Md: Urban and Schwarzenberg; 1981.

LOINC® Map

Order Code Order Code Name Order Loinc Result Code Result Code Name UofM Result LOINC
700140 Amino Acid Profile, Qn, Urine 700069 Taurine umol/g Cr 28595-7
700140 Amino Acid Profile, Qn, Urine 700071 Aspartate umol/g Cr 30061-6
700140 Amino Acid Profile, Qn, Urine 700072 Hydroxyproline umol/g Cr 28601-3
700140 Amino Acid Profile, Qn, Urine 700073 Threonine umol/g Cr 30057-4
700140 Amino Acid Profile, Qn, Urine 700074 Serine umol/g Cr 30058-2
700140 Amino Acid Profile, Qn, Urine 700075 Asparagine umol/g Cr 28603-9
700140 Amino Acid Profile, Qn, Urine 700076 Glutamate umol/g Cr 30059-0
700140 Amino Acid Profile, Qn, Urine 700077 Glutamine umol/g Cr 30056-6
700140 Amino Acid Profile, Qn, Urine 700078 Sarcosine umol/g Cr 28610-4
700140 Amino Acid Profile, Qn, Urine 700079 Alpha-aminoadipate umol/g Cr 28598-1
700140 Amino Acid Profile, Qn, Urine 700080 Proline umol/g Cr 30067-3
700140 Amino Acid Profile, Qn, Urine 700081 Glycine umol/g Cr 30066-5
700140 Amino Acid Profile, Qn, Urine 700082 Alanine umol/g Cr 30068-1
700140 Amino Acid Profile, Qn, Urine 700083 Citrulline umol/g Cr 30161-4
700140 Amino Acid Profile, Qn, Urine 700084 Alpha-aminobutyrate umol/g Cr 28590-8
700140 Amino Acid Profile, Qn, Urine 700085 Valine umol/g Cr 30064-0
700140 Amino Acid Profile, Qn, Urine 700086 Cystine umol/g Cr 30065-7
700140 Amino Acid Profile, Qn, Urine 700087 Methionine umol/g Cr 30063-2
700140 Amino Acid Profile, Qn, Urine 700090 Homocitrulline umol/g Cr 32248-7
700140 Amino Acid Profile, Qn, Urine 700091 Cystathionine umol/g Cr 28599-9
700140 Amino Acid Profile, Qn, Urine 700092 Alloisoleucine umol/g Cr 73908-6
700140 Amino Acid Profile, Qn, Urine 700093 Isoleucine umol/g Cr 30052-5
700140 Amino Acid Profile, Qn, Urine 700094 Leucine umol/g Cr 30053-3
700140 Amino Acid Profile, Qn, Urine 700095 Tyrosine umol/g Cr 30054-1
700140 Amino Acid Profile, Qn, Urine 700098 Phenylalanine umol/g Cr 30055-8
700140 Amino Acid Profile, Qn, Urine 700099 Argininosuccinate umol/g Cr 32229-7
700140 Amino Acid Profile, Qn, Urine 700100 Beta-alanine umol/g Cr 28588-2
700140 Amino Acid Profile, Qn, Urine 700101 Beta-aminoisobutyrate umol/g Cr 28602-1
700140 Amino Acid Profile, Qn, Urine 700102 Homocystine umol/g Cr 30051-7
700140 Amino Acid Profile, Qn, Urine 700103 Gamma-aminobutyrate umol/g Cr 28593-2
700140 Amino Acid Profile, Qn, Urine 700106 Tryptophan umol/g Cr 28608-8
700140 Amino Acid Profile, Qn, Urine 700107 Hydroxylysine umol/g Cr 30050-9
700140 Amino Acid Profile, Qn, Urine 700108 Ornithine umol/g Cr 30049-1
700140 Amino Acid Profile, Qn, Urine 700109 Lysine umol/g Cr 30048-3
700140 Amino Acid Profile, Qn, Urine 700110 Histidine umol/g Cr 30047-5
700140 Amino Acid Profile, Qn, Urine 700111 Arginine umol/g Cr 30062-4
700140 Amino Acid Profile, Qn, Urine 700133 Interpretation 49248-8
700140 Amino Acid Profile, Qn, Urine 700134 Director Review 72486-4
700140 Amino Acid Profile, Qn, Urine 700135 Methodology 49549-9
700140 Amino Acid Profile, Qn, Urine 700471 Disclaimer: N/A
700140 Amino Acid Profile, Qn, Urine 700136 Amino Acid, Ur N/A
Reflex Table for Taurine
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Reflex Table for Aspartate
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Reflex Table for Hydroxyproline
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Reflex Table for Threonine
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Reflex Table for Serine
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Reflex Table for Asparagine
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Reflex Table for Sarcosine
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Reflex Table for Alpha-aminoadipate
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Reflex Table for Proline
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Reflex Table for Glycine
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Reflex Table for Alanine
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Reflex Table for Citrulline
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Reflex Table for Alpha-aminobutyrate
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Reflex Table for Valine
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Reflex Table for Cystine
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Reflex Table for Methionine
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Reflex Table for Homocitrulline
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Reflex Table for Cystathionine
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Reflex Table for Alloisoleucine
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Reflex Table for Isoleucine
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Reflex Table for Leucine
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Reflex Table for Tyrosine
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Reflex Table for Phenylalanine
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Reflex Table for Argininosuccinate
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Reflex Table for Beta-alanine
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Reflex Table for Beta-aminoisobutyrate
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Reflex Table for Homocystine
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Reflex 1 700471 Disclaimer: 700471 Disclaimer: N/A
Reflex Table for Gamma-aminobutyrate
Order Code Order Name Result Code Result Name UofM Result LOINC
Reflex 1 700471 Disclaimer: 700471 Disclaimer: N/A
Reflex Table for Tryptophan
Order Code Order Name Result Code Result Name UofM Result LOINC
Reflex 1 700471 Disclaimer: 700471 Disclaimer: N/A
Reflex Table for Hydroxylysine
Order Code Order Name Result Code Result Name UofM Result LOINC
Reflex 1 700471 Disclaimer: 700471 Disclaimer: N/A
Reflex Table for Ornithine
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Reflex 1 700471 Disclaimer: 700471 Disclaimer: N/A
Reflex Table for Lysine
Order Code Order Name Result Code Result Name UofM Result LOINC
Reflex 1 700471 Disclaimer: 700471 Disclaimer: N/A
Reflex Table for Histidine
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Reflex 1 700471 Disclaimer: 700471 Disclaimer: N/A
Reflex Table for Arginine
Order Code Order Name Result Code Result Name UofM Result LOINC
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Reflex Table for Interpretation
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