--- Query Results ---

Program:Genetics and Genomics
Test Name:Carnitine - Total and Free, blood (G&G South ACH)
Test Code:CARN-P, CARN-S
Performing Site: Alberta Children's Hospital
Performing Dept:Biochemical Genetics
Availability:Test performed on a weekly basis.
TAT:14 days
Preferred Tube/Container:Sodium heparin (dark green - NOT PST)
Alternate:Lithium heparin (dark green - NOT PST)
Non-additive (red top - NOT SST)
Specimen Requirements:1.  Collect 1 x 4.0 mL sodium heparin tube (or alternate tube type).
2.  Collect following a minimum four-hour fast; or just before next feed for newborns.
3.  Indicate length of fast, or time of last feeding on the requisition (with collection time).
Min. Sample Required:0.25 mL plasma or serum
Specimen Processing:At ACH only
The primary heparinized tube can be put on ice (ensure not to freeze sample) or refrigerated for routine pick-ups between 8:30 am – 2:30 pm, Monday through Friday.

For all other collections
1. Centrifuge.
2. Transfer plasma or serum to aliquot tube.
3. Write "NaHep", "LiHep" or "Red Top" on the aliquot tube label.
4. Freeze.
Specimen Handling:Transport frozen samples on ice. Samples must not thaw during transport.
Additional Information:Samples will be rejected if:
  • Primary tube not refrigerated
  • Aliquot tube received thawed
  • Tube type not written on aliquot tube label
    Requisition/Form:Submit request using Biochemical Genetics Laboratory Requisition.
    Please ensure all required information, including patient's clinical history/indication, is provided on the requisition.
    Order Restrictions:None
    Indications:This test is for the investigation of a systemic carnitine deficiency due to an underlying primary (i.e., carnitine uptake defect due to OCTN2 deficiency) or secondary (e.g., MCAD deficiency, organic acidurias) cause.

    This test can also be used to evaluate the potential of a secondary carnitine deficiency due to nutritional inadequacy (e.g., vegan diet, total parenteral nutrition), increased metabolic demand (e.g., ketogenic diet), reduced intestinal absorption (e.g., short bowel syndrome) or increased loss (e.g., Fanconi syndrome, hemodialysis).

    This test can also be used to monitor known metabolic patients on carnitine supplementation therapy.
    Method: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.
    Method Details:Determination of plasma or serum total and free carnitine levels, with calculated acylcarnitine fraction, enables the identification of a systemic carnitine deficiency (i.e., significantly low total and free carnitine levels with or without low acylcarnitine fraction, depending on underlying cause). Other alterations of total, free and/or acyl component measures of the test can be influenced by a patient’s underlying pathology, nutritional status as well as the application of carnitine supplementation therapy, and therefore such information should be provided on the requisition. Follow up testing of abnormal results may include recommendations for evaluation of the patient’s nutritional status, dietary predilections, current treatment and/or assessment of renal function.

    This test may be reflexively performed on a sample in which a result from Acylcarnitine Profile (ACH) is suggestive of low free carnitine (a metabolite monitored but not reported by that test).

    Acylcarnitine Profile (ACH) testing may be reflexibvely performed based on evidence of unexplained increased acylcarnitine accumulation.
    Reference Interval:Provided on test report, based on patient age.
    Comments:Contact ACH Biochemical Genetics Laboratory (403-955-7379) for priority turn-around.
    Last Updated On:Monday, March 26, 2018
    Date of Last Review:Jan 2 2018 12:00AM