Alberta Precision Laboratories

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Organization:

Alberta Precision Laboratories

Message Alert:

PLEASE NOTE: This Test Directory is in an interim state and due to changes pertaining to the DynaLIFE transition, performing site for routing has not been updated. Please refer to Laboratory Information System for appropriate routing.

Test Name/Synonym:

Amino Acids Quantitation, Urine, Random

Clinical Indications:

This test is for the specific investigation of disorders of amino acid transport (e.g., cystinuria, Hartnup disorder) and selected disorders of amino acid metabolism that require confirmation and/or additional metabolite identification/discrimination.

Test Includes:

Metabolites reported from both BCG North and BCG South:

alanine	alpha-aminobutyric acid	arginine
aspartic acid	cystine	glutamic acid
glutamine	glycine	histidine
isoleucine	leucine	lysine
methionine	ornithine	phenylalanine
serine	taurine	threonine
tryptophan	tyrosine	valine

Additional metabolites reported from BCG North:

alloisoleucine

argininosuccinic acid

homocitrulline

Additional metabolites reported from BCG South:

1-methylhistidine	3-methylhistidine	alpha-aminoadipic acid
anserine	asparagine	beta-alanine
beta-aminoisobutyric acid	carnosine	citrulline
cystathionine	ethanolamine	gamma-aminobutyric acid
homocystine	hydroxylysine	hydroxyproline
phosphoethanolamine	phosphoserine	proline
sarcosine

Ordering Alert:

This test is considered specialty testing; this test will be cancelled if sufficient clinical justification is not provided.

This test has limited clinical utility for investigating disorders of amino acid metabolism and should not be used as a frontline test for these disorders. Amino Acids Quantitation, Plasma is recommended as a frontline test.

LABID (Connect Care):

LAB355A

Test Code:

CALGARY ZONE	BGLNON; IN BGL NON-BLOOD TEST NAME FIELD ENTER AMINO ACIDS UR
CENTRAL ZONE	AAUR
EDMONTON ZONE	UAAQ
NORTH ZONE	AAUR
SOUTH ZONE	AAQT

Specimen Type:

Urine

Primary Container:

Sterile container

Alternate Containers:

Non sterile container

Minimum Collection Volume:

1.0 mL

Specimen Collection Requirements:

Collect a 5.0 to 10.0 mL random urine specimen.

Test Resources:

Submit request using the applicable requisition or Connect Care online order entry:

Biochemical Genetics Laboratory Requisition

Please ensure all required information, including patient's clinical history/indication, is provided on the requisition.

Stability and Storage:

Frozen

Processing:

This test requires creatinine determination for result reporting.
For sites using EPIC, a separate aliquot should be removed at the time of initial specimen receipt and sent to the EPIC-designated lab for creatinine testing.

One aliquot with primary container, or two separate aliquots, can be submitted.
Write “urine” on all secondary aliquot tubes.
Freeze container and/or tubes.

Transportation:

Transport frozen specimens on dry ice.
Samples must not thaw during transport.

Method:

Ion exchange liquid chromatography

Method and Interpretation of Results:

Amino acids are detected using an Amino Acid Analyzer with internal standard.

Amino acid analysis enables the diagnosis of specific disorders of amino acid metabolism, as relevant primary enzyme and transporter deficiencies result in abnormal concentrations of specific amino acids. Secondary disorders that caused impaired renal function may result in abnormal amino acid concentrations that can be identified by this test.

Primary Disorders Detectable by Urine Amino Acid Analysis:

alpha-aminoadipic aciduria	argininosuccinic aciduria (argininosuccinate lyase deficiency)
beta-alaninemia	carnosinemia
cystathionase deficiency	cystinuria
dibasic aminoaciduria	dicarboxylic aminoaciduria
disorders of pyrimidine metabolism	familial renal iminoglycinuria
Hartnup disorder	HHH syndrome
hydroxyprolinuria	hyperlysinemia
hyperornithinemia	hyperprolinemia
hypophosphatasia	lysinuric protein intolerance
saccharopinuria	tryptophanuria

Secondary Disorders Detectable by Urine Amino Acid Analysis:

Fanconi syndrome

Vitamin D-dependent rickets

Further confirmatory testing is required for many of these conditions, as nonspecific findings and overlapping patterns may prevent definitive diagnosis in specific cases.

This test is not a reliable method for detecting and quantitating homocystine, as levels can be altered depending on the length of time between sample collection and analysis, as well as the presence of an interfering peak that falsely suggests elevated homocystine. Recommend ordering Homocysteine, Total, Blood (Metabolic Disorders) or Homocysteine, Total, Urine, Random for evaluation of homocytine levels.

Similarly, various medications can lead to ninhydrin-positive peaks that interfere with the identification and quantitation of certain amino acids. It is recommend that all current medications being take at the time of specimen collection be provided in the reason for referral.

Routine Turn Around Time:

14 days

Testing Schedule:

Daily

Reference Interval:

Reference Intervals are age-dependent and normalized by creatinine, and will be provided on the report.

Testing Area:

Biochemical Genetics

Performing Site:

University of Alberta Hospital or Alberta Children’s Hospital

Additional Test Information:

For priority turn-around contact Biochemical Genetics Laboratory.

Contact Comments:

Biochemical Genetic North	Biochemical Genetic South
780-407-8820	403-955-7379

Last Updated On:

Tuesday, November 22, 2022

Date of Last Review:

Aug 1 2022 12:00AM