700140: Amino Acid Profile, Quantitative, Urine

Test Details

Methodology

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

Result Turnaround Time

3 - 7 days

Turnaround time is defined as the usual number of days from the date of pickup of a specimen for testing to when the result is released to the ordering provider. In some cases, additional time should be allowed for additional confirmatory or additional reflex tests. Testing schedules may vary.

Related Information

Amino Acid Test Options

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 Food and Drug Administration.

References

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

Specimen Requirements

Specimen

Urine, frozen

Volume

5 mL

Minimum Volume

0.2 mL (Note: This volume does not allow for repeat testing.)

Container

Plastic urine container without preservative

Stability Requirements

Temperature	Period
Room temperature	Unstable
Refrigerated	Unstable
Frozen	14 days

Reference Range

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

Storage Instructions

Freeze.

Causes for Rejection

Room temperature or thawed specimens

LOINC® Map

Order Code	Order Code Name	Order Loinc	Result Code	Result Code Name	UofM	Result LOINC
700140	Amino Acid Profile, Qn, Urine	35087-6	700069	Taurine	umol/g Cr	28595-7
700140	Amino Acid Profile, Qn, Urine	35087-6	700071	Aspartate	umol/g Cr	30061-6
700140	Amino Acid Profile, Qn, Urine	35087-6	700072	Hydroxyproline	umol/g Cr	28601-3
700140	Amino Acid Profile, Qn, Urine	35087-6	700073	Threonine	umol/g Cr	30057-4
700140	Amino Acid Profile, Qn, Urine	35087-6	700074	Serine	umol/g Cr	30058-2
700140	Amino Acid Profile, Qn, Urine	35087-6	700075	Asparagine	umol/g Cr	28603-9
700140	Amino Acid Profile, Qn, Urine	35087-6	700076	Glutamate	umol/g Cr	30059-0
700140	Amino Acid Profile, Qn, Urine	35087-6	700077	Glutamine	umol/g Cr	30056-6
700140	Amino Acid Profile, Qn, Urine	35087-6	700078	Sarcosine	umol/g Cr	28610-4
700140	Amino Acid Profile, Qn, Urine	35087-6	700079	Alpha-aminoadipate	umol/g Cr	28598-1
700140	Amino Acid Profile, Qn, Urine	35087-6	700080	Proline	umol/g Cr	30067-3
700140	Amino Acid Profile, Qn, Urine	35087-6	700081	Glycine	umol/g Cr	30066-5
700140	Amino Acid Profile, Qn, Urine	35087-6	700082	Alanine	umol/g Cr	30068-1
700140	Amino Acid Profile, Qn, Urine	35087-6	700083	Citrulline	umol/g Cr	30161-4
700140	Amino Acid Profile, Qn, Urine	35087-6	700084	Alpha-aminobutyrate	umol/g Cr	28590-8
700140	Amino Acid Profile, Qn, Urine	35087-6	700085	Valine	umol/g Cr	30064-0
700140	Amino Acid Profile, Qn, Urine	35087-6	700086	Cystine	umol/g Cr	30065-7
700140	Amino Acid Profile, Qn, Urine	35087-6	700087	Methionine	umol/g Cr	30063-2
700140	Amino Acid Profile, Qn, Urine	35087-6	700090	Homocitrulline	umol/g Cr	32248-7
700140	Amino Acid Profile, Qn, Urine	35087-6	700091	Cystathionine	umol/g Cr	28599-9
700140	Amino Acid Profile, Qn, Urine	35087-6	700092	Alloisoleucine	umol/g Cr	73908-6
700140	Amino Acid Profile, Qn, Urine	35087-6	700093	Isoleucine	umol/g Cr	30052-5
700140	Amino Acid Profile, Qn, Urine	35087-6	700094	Leucine	umol/g Cr	30053-3
700140	Amino Acid Profile, Qn, Urine	35087-6	700095	Tyrosine	umol/g Cr	30054-1
700140	Amino Acid Profile, Qn, Urine	35087-6	700098	Phenylalanine	umol/g Cr	30055-8
700140	Amino Acid Profile, Qn, Urine	35087-6	700099	Argininosuccinate	umol/g Cr	32229-7
700140	Amino Acid Profile, Qn, Urine	35087-6	700100	Beta-alanine	umol/g Cr	28588-2
700140	Amino Acid Profile, Qn, Urine	35087-6	700101	Beta-aminoisobutyrate	umol/g Cr	28602-1
700140	Amino Acid Profile, Qn, Urine	35087-6	700102	Homocystine	umol/g Cr	30051-7
700140	Amino Acid Profile, Qn, Urine	35087-6	700103	Gamma-aminobutyrate	umol/g Cr	28593-2
700140	Amino Acid Profile, Qn, Urine	35087-6	700106	Tryptophan	umol/g Cr	28608-8
700140	Amino Acid Profile, Qn, Urine	35087-6	700107	Hydroxylysine	umol/g Cr	30050-9
700140	Amino Acid Profile, Qn, Urine	35087-6	700108	Ornithine	umol/g Cr	30049-1
700140	Amino Acid Profile, Qn, Urine	35087-6	700109	Lysine	umol/g Cr	30048-3
700140	Amino Acid Profile, Qn, Urine	35087-6	700110	Histidine	umol/g Cr	30047-5
700140	Amino Acid Profile, Qn, Urine	35087-6	700111	Arginine	umol/g Cr	30062-4
700140	Amino Acid Profile, Qn, Urine	35087-6	700133	Interpretation		49248-8
700140	Amino Acid Profile, Qn, Urine	35087-6	700134	Director Review		72486-4
700140	Amino Acid Profile, Qn, Urine	35087-6	700135	Methodology		49549-9
Order Code	700140
Order Code Name	Amino Acid Profile, Qn, Urine
Order Loinc	35087-6
Result Code	700069
Result Code Name	Taurine
UofM	umol/g Cr
Result LOINC	28595-7
Order Code	700140
Order Code Name	Amino Acid Profile, Qn, Urine
Order Loinc	35087-6
Result Code	700071
Result Code Name	Aspartate
UofM	umol/g Cr
Result LOINC	30061-6
Order Code	700140
Order Code Name	Amino Acid Profile, Qn, Urine
Order Loinc	35087-6
Result Code	700072
Result Code Name	Hydroxyproline
UofM	umol/g Cr
Result LOINC	28601-3
Order Code	700140
Order Code Name	Amino Acid Profile, Qn, Urine
Order Loinc	35087-6
Result Code	700073
Result Code Name	Threonine
UofM	umol/g Cr
Result LOINC	30057-4
Order Code	700140
Order Code Name	Amino Acid Profile, Qn, Urine
Order Loinc	35087-6
Result Code	700074
Result Code Name	Serine
UofM	umol/g Cr
Result LOINC	30058-2
Order Code	700140
Order Code Name	Amino Acid Profile, Qn, Urine
Order Loinc	35087-6
Result Code	700075
Result Code Name	Asparagine
UofM	umol/g Cr
Result LOINC	28603-9
Order Code	700140
Order Code Name	Amino Acid Profile, Qn, Urine
Order Loinc	35087-6
Result Code	700076
Result Code Name	Glutamate
UofM	umol/g Cr
Result LOINC	30059-0
Order Code	700140
Order Code Name	Amino Acid Profile, Qn, Urine
Order Loinc	35087-6
Result Code	700077
Result Code Name	Glutamine
UofM	umol/g Cr
Result LOINC	30056-6
Order Code	700140
Order Code Name	Amino Acid Profile, Qn, Urine
Order Loinc	35087-6
Result Code	700078
Result Code Name	Sarcosine
UofM	umol/g Cr
Result LOINC	28610-4
Order Code	700140
Order Code Name	Amino Acid Profile, Qn, Urine
Order Loinc	35087-6
Result Code	700079
Result Code Name	Alpha-aminoadipate
UofM	umol/g Cr
Result LOINC	28598-1
Order Code	700140
Order Code Name	Amino Acid Profile, Qn, Urine
Order Loinc	35087-6
Result Code	700080
Result Code Name	Proline
UofM	umol/g Cr
Result LOINC	30067-3
Order Code	700140
Order Code Name	Amino Acid Profile, Qn, Urine
Order Loinc	35087-6
Result Code	700081
Result Code Name	Glycine
UofM	umol/g Cr
Result LOINC	30066-5
Order Code	700140
Order Code Name	Amino Acid Profile, Qn, Urine
Order Loinc	35087-6
Result Code	700082
Result Code Name	Alanine
UofM	umol/g Cr
Result LOINC	30068-1
Order Code	700140
Order Code Name	Amino Acid Profile, Qn, Urine
Order Loinc	35087-6
Result Code	700083
Result Code Name	Citrulline
UofM	umol/g Cr
Result LOINC	30161-4
Order Code	700140
Order Code Name	Amino Acid Profile, Qn, Urine
Order Loinc	35087-6
Result Code	700084
Result Code Name	Alpha-aminobutyrate
UofM	umol/g Cr
Result LOINC	28590-8
Order Code	700140
Order Code Name	Amino Acid Profile, Qn, Urine
Order Loinc	35087-6
Result Code	700085
Result Code Name	Valine
UofM	umol/g Cr
Result LOINC	30064-0
Order Code	700140
Order Code Name	Amino Acid Profile, Qn, Urine
Order Loinc	35087-6
Result Code	700086
Result Code Name	Cystine
UofM	umol/g Cr
Result LOINC	30065-7
Order Code	700140
Order Code Name	Amino Acid Profile, Qn, Urine
Order Loinc	35087-6
Result Code	700087
Result Code Name	Methionine
UofM	umol/g Cr
Result LOINC	30063-2
Order Code	700140
Order Code Name	Amino Acid Profile, Qn, Urine
Order Loinc	35087-6
Result Code	700090
Result Code Name	Homocitrulline
UofM	umol/g Cr
Result LOINC	32248-7
Order Code	700140
Order Code Name	Amino Acid Profile, Qn, Urine
Order Loinc	35087-6
Result Code	700091
Result Code Name	Cystathionine
UofM	umol/g Cr
Result LOINC	28599-9
Order Code	700140
Order Code Name	Amino Acid Profile, Qn, Urine
Order Loinc	35087-6
Result Code	700092
Result Code Name	Alloisoleucine
UofM	umol/g Cr
Result LOINC	73908-6
Order Code	700140
Order Code Name	Amino Acid Profile, Qn, Urine
Order Loinc	35087-6
Result Code	700093
Result Code Name	Isoleucine
UofM	umol/g Cr
Result LOINC	30052-5
Order Code	700140
Order Code Name	Amino Acid Profile, Qn, Urine
Order Loinc	35087-6
Result Code	700094
Result Code Name	Leucine
UofM	umol/g Cr
Result LOINC	30053-3
Order Code	700140
Order Code Name	Amino Acid Profile, Qn, Urine
Order Loinc	35087-6
Result Code	700095
Result Code Name	Tyrosine
UofM	umol/g Cr
Result LOINC	30054-1
Order Code	700140
Order Code Name	Amino Acid Profile, Qn, Urine
Order Loinc	35087-6
Result Code	700098
Result Code Name	Phenylalanine
UofM	umol/g Cr
Result LOINC	30055-8
Order Code	700140
Order Code Name	Amino Acid Profile, Qn, Urine
Order Loinc	35087-6
Result Code	700099
Result Code Name	Argininosuccinate
UofM	umol/g Cr
Result LOINC	32229-7
Order Code	700140
Order Code Name	Amino Acid Profile, Qn, Urine
Order Loinc	35087-6
Result Code	700100
Result Code Name	Beta-alanine
UofM	umol/g Cr
Result LOINC	28588-2
Order Code	700140
Order Code Name	Amino Acid Profile, Qn, Urine
Order Loinc	35087-6
Result Code	700101
Result Code Name	Beta-aminoisobutyrate
UofM	umol/g Cr
Result LOINC	28602-1
Order Code	700140
Order Code Name	Amino Acid Profile, Qn, Urine
Order Loinc	35087-6
Result Code	700102
Result Code Name	Homocystine
UofM	umol/g Cr
Result LOINC	30051-7
Order Code	700140
Order Code Name	Amino Acid Profile, Qn, Urine
Order Loinc	35087-6
Result Code	700103
Result Code Name	Gamma-aminobutyrate
UofM	umol/g Cr
Result LOINC	28593-2
Order Code	700140
Order Code Name	Amino Acid Profile, Qn, Urine
Order Loinc	35087-6
Result Code	700106
Result Code Name	Tryptophan
UofM	umol/g Cr
Result LOINC	28608-8
Order Code	700140
Order Code Name	Amino Acid Profile, Qn, Urine
Order Loinc	35087-6
Result Code	700107
Result Code Name	Hydroxylysine
UofM	umol/g Cr
Result LOINC	30050-9
Order Code	700140
Order Code Name	Amino Acid Profile, Qn, Urine
Order Loinc	35087-6
Result Code	700108
Result Code Name	Ornithine
UofM	umol/g Cr
Result LOINC	30049-1
Order Code	700140
Order Code Name	Amino Acid Profile, Qn, Urine
Order Loinc	35087-6
Result Code	700109
Result Code Name	Lysine
UofM	umol/g Cr
Result LOINC	30048-3
Order Code	700140
Order Code Name	Amino Acid Profile, Qn, Urine
Order Loinc	35087-6
Result Code	700110
Result Code Name	Histidine
UofM	umol/g Cr
Result LOINC	30047-5
Order Code	700140
Order Code Name	Amino Acid Profile, Qn, Urine
Order Loinc	35087-6
Result Code	700111
Result Code Name	Arginine
UofM	umol/g Cr
Result LOINC	30062-4
Order Code	700140
Order Code Name	Amino Acid Profile, Qn, Urine
Order Loinc	35087-6
Result Code	700133
Result Code Name	Interpretation
UofM
Result LOINC	49248-8
Order Code	700140
Order Code Name	Amino Acid Profile, Qn, Urine
Order Loinc	35087-6
Result Code	700134
Result Code Name	Director Review
UofM
Result LOINC	72486-4
Order Code	700140
Order Code Name	Amino Acid Profile, Qn, Urine
Order Loinc	35087-6
Result Code	700135
Result Code Name	Methodology
UofM
Result LOINC	49549-9

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