--- Query Results --- | |||||||||||||||||||||||||||||||||||||
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: | Acylcarnitine Profile, Blood | ||||||||||||||||||||||||||||||||||||
Clinical Indications: | This test is for the investigation of mitochondrial fatty acid oxidation disorders and certain organic acidurias.
This test may also be ordered to rule out the possibility of these disorders in a patient being assessed for the ketogenic diet. This test may also be used to monitor known patients with certain mitochondrial fatty acid oxidation disorders or organic acidurias on diet-modified treatment. | ||||||||||||||||||||||||||||||||||||
Test Includes: |
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Collection Alert: | Hours fasting must be indicated. Recommend collection following a minimum four hour fast or just before next feeding for newborns. Specimens collected from non-fasting patients will be accepted for testing. | ||||||||||||||||||||||||||||||||||||
LABID (Connect Care): | LAB4350 | ||||||||||||||||||||||||||||||||||||
Test Code: |
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Specimen Type: | Blood | ||||||||||||||||||||||||||||||||||||
Primary Container: | Green top – Sodium heparin (no gel) | ||||||||||||||||||||||||||||||||||||
Alternate Containers: | Green top – Lithium heparin (no gel) Red top – Non-additive (no gel) | ||||||||||||||||||||||||||||||||||||
Minimum Collection Volume: | 0.5 mL plasma or serum | ||||||||||||||||||||||||||||||||||||
Specimen Collection Requirements: | Collect one Sodium Heparin (or alternate) tube. Indicate length of fast, or time of last feeding on the requisition (with collection time). | ||||||||||||||||||||||||||||||||||||
Test Resources: | Submit request using the applicable requisition or Connect Care online order entry:
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Stability and Storage: | Frozen: plasma or serum aliquot | ||||||||||||||||||||||||||||||||||||
Processing: | For ALL collection locations except for Alberta Children’s Hospital (ACH): 1. Centrifuge; If red top tube is collected, allow to fully clot prior to centrifugation. 2. Transfer plasma or serum to aliquot tube. 3. Write ""NaHep"", ""LiHep"" or ""Red Top"" on the aliquot tube label. 4. Freeze aliquot tube. If collected at ACH outpatient Lab:
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Transportation: | Transport frozen specimens on dry ice. Samples must not thaw during transport. | ||||||||||||||||||||||||||||||||||||
Method: | Liquid chromatography tandem mass spectrometry (LC-MS/MS) | ||||||||||||||||||||||||||||||||||||
Method and Interpretation of Results: | Acylcarnitines are detected following derivatization (i.e., butylation) using electrospray ionization tandem mass spectrometry (ESI-MS/MS) with deuterium-labelled internal standards.
Acylcarnitine analysis enables the diagnosis of specific disorders of fatty acid oxidation and certain organic acidurias, as relevant primary enzyme deficiencies result in the accumulation of specific acylcarnitines. Disorders Detectable by Acylcarnitine Analysis: 1. Fatty Acid Oxidation Disorders:
Free carnitine is not reported as part of this test; therefore Carnitine, Total and Free - Blood (ACH) should be ordered separately if there is suspicion of a systemic carnitine deficiency. Urine organic acids will be reflexively performed on any existing sample if the results of the plasma acylcarnitine indicate the need for further investigation or metabolite discrimination. | ||||||||||||||||||||||||||||||||||||
Routine Turn Around Time: | 14 days | ||||||||||||||||||||||||||||||||||||
Testing Schedule: | Weekly | ||||||||||||||||||||||||||||||||||||
Reference Interval: | Provided on test report based on patient age. | ||||||||||||||||||||||||||||||||||||
Testing Area: | Biochemical Genetics | ||||||||||||||||||||||||||||||||||||
Performing Site: | Alberta Children's Hospital | ||||||||||||||||||||||||||||||||||||
Additional Test Information: | For priority turn-around contact Biochemical Genetics South Laboratory. | ||||||||||||||||||||||||||||||||||||
Contact Comments: | Biochemical Genetics 403-955-7379 | ||||||||||||||||||||||||||||||||||||
Last Updated On: | Monday, July 24, 2023 | ||||||||||||||||||||||||||||||||||||
Date of Last Review: | Aug 1 2022 12:00AM | ||||||||||||||||||||||||||||||||||||