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Test Code AAMSD Amino Acids, Maple Syrup Urine Disease Panel, Plasma

Performing Laboratory

Mayo Clinic Laboratories in Rochester

Specimen Type

Plasma


Ordering Guidance


Body fluids are not acceptable specimens for this test.

For testing urine specimens, order AAPD / Amino Acids, Quantitative, Random, Urine.

For testing spinal fluid specimens, order AACSF / Amino Acids, Quantitative, Spinal Fluid.



Necessary Information


1. Patient's age is required.

2. Include family history, clinical condition (asymptomatic or acute episode), diet, and drug therapy information.



Specimen Required


Patient Preparation: Patient should fast overnight (4 hours minimum); infants should have specimen collected before next feeding (2-3 hours without total parenteral nutrition if possible).

Supplies: Sarstedt Aliquot Tube, 5 mL (T914)

Collection Container/Tube: Green top (sodium heparin)

Submission Container/Tube: Plastic vial

Specimen Volume: 0.5 mL

Collection Instructions:

1. Collect specimen and place on wet ice. Note: Thrombin-activated tubes should not be used for collection.

2. Centrifuge immediately or within 4 hours of collection if specimen is kept at refrigerated temperature.

3. Being careful to ensure that no buffy coat is transferred, aliquot plasma into a plastic vial and freeze.


Specimen Minimum Volume

0.3 mL

Specimen Stability Information

Specimen Type Temperature Time Special Container
Plasma Frozen 14 days

Reference Values

Isoleucine

<24 months: 23-149 nmol/mL

2-17 years: 26-150 nmol/mL

≥18 years: 29-153 nmol/mL

 

Leucine

<24 months: 59-213 nmol/mL

2-17 years: 51-216 nmol/mL

≥18 years: 79-217 nmol/mL

 

Valine

<24 months: 94-382 nmol/mL

2-17 years: 111-367 nmol/mL

≥18 years: 134-357 nmol/mL

 

Allo-isoleucine

<5 nmol/mL

Day(s) Performed

Monday through Friday

CPT Code Information

82136

Report Available

3 to 5 days

Specimen Retention Time

2 weeks

Reject Due To

Gross hemolysis OK
Gross lipemia OK
Gross icterus OK