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TEST ID MMPP Mitochondrial Metabolites, Plasma

Reporting Name

Mitochondrial Metabolites, P

Specimen Type

Plasma


Ordering Guidance


This test is not the recommended initial screening test for evaluating patients with suspected mitochondrial disorders, organic acidurias, and ketone body disorders. For these purposes, the preferred tests for first-tier assessment are OAU / Organic Acids Screen, Random, Urine; AAQP / Amino Acids, Quantitative, Plasma; and ACRN / Acylcarnitines, Quantitative, Plasma.

 

Analytes from LAPYP / Lactate Pyruvate Panel, Plasma are included in this test. If ordered together, LAPYP may be canceled.



Specimen Required


Collection Container/Tube:

Preferred: Green top (Sodium heparin)

Acceptable: Green top (Lithium heparin)

Submission Container/Tube: Plastic vial

Specimen Volume: 0.5 mL

Collection Instructions: Centrifuge and aliquot plasma into a plastic vial.


Specimen Minimum Volume

0.1 mL

Specimen Stability Information

Specimen Type Temperature Time Special Container
Plasma Frozen 7 days

Method Name

Gas Chromatography Mass Spectrometry (GC-MS)

Reject Due To

Gross hemolysis Reject
Gross lipemia OK
Gross icterus OK

Reference Values

LACTIC ACID

≤ 4000.0 nmol/mL

 

2-HYDROXYBUTYRIC ACID

≤ 124.0 nmol/mL

 

3-HYDROXYBUTYRIC ACID

≤ 700.0 nmol/mL

 

PYRUVIC ACID

≤ 350.0 nmol/mL

 

cis-ACONITIC ACID

≤ 9.0 nmol/mL

 

CITRIC ACID

≤ 250.0 nmol/mL

 

3-HYDROXYPROPIONIC ACID

≤ 12.4 nmol/mL

 

3-HYDROXY-2-METHYLBUTYRIC ACID

≤ 2.5 nmol/mL

 

3-HYDROXYISOVALERIC ACID

≤ 15.4 nmol/mL

 

SUCCINIC ACID

≤ 10.0 nmol/mL

 

FUMARIC ACID

≤ 5.0 nmol/mL

 

3-METHYLGLUTACONIC ACID

≤ 1.6 nmol/mL

 

MALIC ACID

≤ 20.0 nmol/mL

 

2-KETOBUTYRIC ACID

≤ 16.0 nmol/mL

 

2-KETOISOVALERIC ACID

≤ 35.0 nmol/mL

 

ACETOACETIC ACID

≤ 350.0 nmol/mL

 

3-METHYL-2-KETOVALERIC ACID

≤ 70.0 nmol/mL

 

2-KETOISOCAPROIC ACID

≤ 70.0 nmol/mL

 

2-METHYLCITRIC ACID

≤ 1.0 nmol/mL

 

2-KETOGLUTARIC ACID

≤ 40.0 nmol/mL

Day(s) Performed

Wednesday

Report Available

3 to 9 days

Specimen Retention Time

2 months

Performing Laboratory

Mayo Clinic Laboratories in Rochester

CPT Code Information

82542

Forms

If not ordering electronically, complete, print, and send a Biochemical Genetics Test Request (T798) with the specimen.

Useful For

Monitoring patients with mitochondrial disorders, organic acidurias, and ketone body disorders