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Test Code:
090-21-1451-01

Order Name:
Carnitine (Urine) **

 
Useful For:

1. Evaluation of patients with a clinical suspicion of a wide range of conditions including organic acidemias and fatty acid oxidation disorders.
2. Monitoring carnitine treatment.

 
Methodology:
Flow Injection Analysis-Tandem Mass Spectrometry (FIA-MS/MS)
 
AliasesName:
Urinary Carnitine, Quantitative
L-Carnitine
Carnitine Free and Total, Urine
 
 
 
Test Code:
090-21-1451-01

Order Name:
Carnitine (Urine) **

 
Collection Specimen Or Container:
Urine, Clean container 

 
 
Specimen Testing Type:
Urine, minimum volume 20 mL
 
Sub Mission Container:
Clean container 
 
Rejection Criteria:


 
 
Specimen Stabillity:
Specimen Type Temperature Time
Urine Frozen (preferred) 365 days
  Refrigerated 72 hours

 
 
 
 
Test Code:
090-21-1451-01

Order Name:
Carnitine (Urine) **

 
Method detail:
Flow Injection Analysis-Tandem Mass Spectrometry (FIA-MS/MS)
 
Schedule:
N/A **Sent out to MAYO, USA
 
Turnaround Time:
Received specimen to reported within 14 days
 
 
Performing Location:
MAYO Laboratory
Referral Lab Services, Laboratory Department 14160-2
 
 
 
Test Code:
090-21-1451-01

Order Name:
Carnitine (Urine) **

 
 
Clinical Information:

Carnitine and its esters are required for normal energy metabolism and serve 4 primary functions:

-Importing long-chain fatty acids into the mitochondria

-Exporting naturally occurring short-chain acyl-CoA groups from the mitochondria

-Maintaining the ratio of free CoA to esterified CoA

-Removing potentially toxic acyl-CoA groups from the cells and tissues
Evaluation of carnitine in serum, plasma, and urine screens patients for suspected primary disorders of the carnitine cycle, or secondary disturbances in carnitine levels as a result of organic acidemias and fatty acid oxidation disorders. In the latter, acyl-CoA groups accumulate and are excreted into the urine and bile as carnitine derivatives, resulting in a secondary carnitine deficiency. More than 100 such primary and secondary disorders have been described. Collectively, their incidence is approximately 1 in 1,000 live births. Primary carnitine deficiency has an incidence of approximately 1 in 21,000 live births based on Minnesota newborn screening data.
Other conditions that could cause an abnormal carnitine level include neuromuscular diseases, gastrointestinal disorders, familial cardiomyopathy, renal tubulopathies and chronic renal failure (dialysis), and prolonged treatment with steroids, antibiotics (pivalic acid), anticonvulsants (valproic acid), and total parenteral nutrition.
Follow-up testing is required to differentiate primary and secondary carnitine deficiencies and to elucidate the exact cause.

 
Reference Value:

FREE : 77-214 nmol/mg of creatinine
TOTAL : 180-412 nmol/mg of creatinine
RATIO : Acyl to free: 0.7-3.4

 
Interpretation:

When abnormal results are detected, a detailed interpretation is given, including an overview of the results and of their significance, a correlation to available clinical information, elements of differential diagnosis, recommendations for additional biochemical testing and a phone number to reach one of the laboratory directors in case the referring physician has additional questions.

 
Clinical Reference:
www.mayomedicallaboratories.com (Retrieved: 24 Jun 2020)