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:

Carnitine, Total and Free, Urine (Synonyms: Carnitine Deficiency, Carnitine Uptake Defect)

Clinical Indications:

This test is for the further investigation of a patient with clear evidence of a systemic carnitine deficiency based on low plasma/serum total and free carnitine levels, to determine whether the cause of their deficiency is due to specific renal loss (i.e., carnitine uptake defect) or generalized renal insufficiency. Differentiating between primary and secondary cause can be evaluated by calculating fractional carnitine excretion.

Test Includes:

Metabolites reported: Total, Free and Acyl Carnitine

Ordering Alert:

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

This test should always be ordered concurrently with Carnitine, Total and Free, Blood (as the interpretation of these findings is dependent on the patient's current plasma or serum levels), and only following previous findings of abnormal plasma/serum levels.

Collection Alert:

This test should always be collected concurrently with Carnitine, Total and Free, Blood

LABID (Connect Care):

LAB4015A

Test Code:

CALGARY ZONE	BGLBL; IN BGL TEST NAME FIELD ENTER CARNITINE
OTHER ZONES	NOT SPECIFIED

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:

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

Method and Interpretation of Results:

Total and free carnitine levels are measured with and without acid hydrolysis, and the acylcarnitine fraction calculated, using Liquid Chromatography, Electrospray-Ionization Tandem Mass Spectrometry (LC-MS/MS) with a deuterium-labelled internal standard.

Determination of urine total and free carnitine levels enables the confirmation of a systemic carnitine deficiency due to renal loss, and can be influenced by current nutritional status and the application of carnitine supplementation therapy. Therefore, such information should be provided on the requisition.

Routine Turn Around Time:

30 days

Testing Schedule:

Biweekly

Reference Interval:

Metabolite	Noraml Range	Units
Total Carnitine	20.4 - 46.6	mmol/mol creatinine
Free Carnitine	8.7 - 24.2	mmol/mol creatinine
Acyl/Free Carnitine	0.7 - 3.4	ratio

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, August 29, 2022

Date of Last Review:

Aug 1 2022 12:00AM