1-Methylhistidine; 3-Methylhistidine; Alanine; Alloisoleucine; Alpha-aminoadipic acid; Alpha-amino-n-butyric acid; Amino Acids Comment; Anserine; Arginine; Asparagine; Aspartate; Beta-Alanine; Beta-aminoisobutyric acid; Carnosine; Citrulline; Cystathionine; Cystine; Ethanolamine/ARS/Saccharopine; GABA/Homocitrulline; Glutamate; Glutamine; Glycine; Histadine; Hydroxylysine-1; Hydroxylysine-2; Hydroxyproline; Isoleucine; Leucine; Lysine; Methionine; Ornithine; Phenylalanine; Phosphoethanolamine; Phosphoserine; Proline; Sarcosine; Serine; Taurine; Threonine; Tryptophan; Tyrosine; Valine
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Amino Acids, Serum/Plasma and CSF
Test ID: AAQPRO
Test
Method
UPLC absorbance
Report Includes
Specimens
CSF and Serum/Plasma
Clinical Utility
For cerebral aminoacidopathies, particularly defects affecting the glycine cleavage enzyme (nonketotic hyperglycemia (NKH)) and serine deficiency.
Serum/plasma and CSF are required for interpretation of results. Note that fewer amino acids are tested in the CSF specimen – refer to the sample report.
Test Location
Hospital for Sick Children, 555 University Avenue, Toronto ON M5G 1X8
Test Version
11-Dec-2023
Specimen
Specimens
CSF and Serum/Plasma
Collection Containers
Preferred
CSF: Sterile Container
Plasma: Green Top (Li Heparin); Green top (Na Heparin)
Acceptable
Red Top (no additive)
Sample Volume
CSF: 0.5 mL, Serum/Plasma: 1mL
Minimum Volume
CSF: 150 uL, Serum/Plasma: 200 uL
Collection & Handling
Collection Instructions
CSF and serum/plasma specimens must be collected within an hour of each other to facilitate interpretation of findings.
Handling Information
CSF: If sample is clear and free of red cells, freeze immediately in clean tube or vial. If sample is contaminated with red cells, spin down and transfer the clear supernatant to a clean tube or vial before freezing. Store and send frozen.
Serum/plasma: Separate and freeze within 1 hour. Store and send frozen.
Additional Information
Gel separators are not acceptable.
Stability
| Frozen | 30 days |
|---|
Additional Stability Information
CSF: If specimen thaws, it is unsuitable for analysis
Rejection Criteria
| Hemolysis | Present |
|---|---|
| Specimen | Not frozen and gel separators. |
Test Version
11-Dec-2023
Performance / Interpretation
Method
UPLC absorbance
Turnaround Time
8 days
Results
| Name | Units | Reference Range | Conversion Factor | |
|---|---|---|---|---|
| Amino Acids (Various) | µmol/L |
|
||
Sample Reports
Test Location
Hospital for Sick Children, 555 University Avenue, Toronto ON M5G 1X8
Test Version
11-Dec-2023
Interface / Setup
HL7 Interface Codes
| Order Code | Result Codes | Units |
|---|---|---|
| AAQPRO | 62350Phosphoserine | umol/L |
| 62351Hydroxyproline | umol/L | |
| 22843Histidine | umol/L | |
| 62352Phosphoethanolamine | umol/L | |
| 62353Asparagine | umol/L | |
| 623541-Methylhistidine | umol/L | |
| 62355Taurine | umol/L | |
| 623563-Methylhistidine | umol/L | |
| 22851Serine | umol/L | |
| 22855Glutamine | umol/L | |
| 62357Carnosine | umol/L | |
| 22837Arginine | umol/L | |
| 22842Glycine | umol/L | |
| 62358Anserine | umol/L | |
| 62359Ethanolamine/ARS/Saccharopine | umol/L | |
| 22838Aspartate | umol/L | |
| 62360Sarcosine | umol/L | |
| 22841Glutamate | umol/L | |
| 22839Citrulline | umol/L | |
| 62361Beta-Alanine | umol/L | |
| 22852Threonine | umol/L | |
| 22836Alanine | umol/L | |
| 62362GABA/Homocitrulline | umol/L | |
| 62363Alpha-aminoadipic acid | umol/L | |
| 22850Proline | umol/L | |
| 62364Beta-aminoisobutyric acid | umol/L | |
| 62365Hydroxyylysine 1 | umol/L | |
| 62366Hydroxylysine 2 | umol/L | |
| 62367Alpha-amino-n-butyric acid | umol/L | |
| 62368Cystathionine | umol/L | |
| 22848Ornithine | umol/L | |
| 22840Cystine | umol/L | |
| 22846Lysine | umol/L | |
| 22853Tyrosine | umol/L | |
| 22847Methionine | umol/L | |
| 22854Valine | umol/L | |
| 22844Isoleucine | umol/L | |
| 39540Alloisoleucine | umol/L | |
| 22845Leucine | umol/L | |
| 22849Phenylalanine | umol/L | |
| 62369Tryptophan | umol/L | |
| 39544Amino Acid Comment | ||
| 62917Histidine, CSF | umol/L | |
| 62918Asparagine, CSF | umol/L | |
| 62919Taurine, CSF | umol/L | |
| 62920Serine, CSF | umol/L | |
| 62921Glutamine, CSF | umol/L | |
| 62925Citrulline, CSF | umol/L | |
| 62926Threonine, CSF | umol/L | |
| 62927Alanine, CSF | umol/L | |
| 62928Proline, CSF | umol/L | |
| 62929Alpha-amino-n-butyric acid, CSF | umol/L | |
| 62930Ornithine, CSF | umol/L | |
| 62931Cystine, CSF | umol/L | |
| 62932Lysine, CSF | umol/L | |
| 62933Tyrosine, CSF | umol/L | |
| 62934Methionine, CSF | umol/L | |
| 62935Valine, CSF | umol/L | |
| 62936Isoleucine, CSF | umol/L | |
| 62937Leucine, CSF | umol/L | |
| 62938Phenylalanine, CSF | umol/L | |
| 62939Tryptophan, CSF | umol/L | |
| 62563Amino Acids CSF Comment |
Sample Reports
Test Version
11-Dec-2023