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Test Code LAB1231911 Amino Acids, Maple Syrup Urine Disease Panel, Plasma

Important Note

Mayo code is AAMSD

Performing Laboratory

Mayo Clinic Laboratories in Rochester

Reporting Name

Amino Acid, MSUD Panel, P

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.


Reject Due To

Gross hemolysis OK
Gross lipemia OK
Gross icterus OK

Specimen Stability Information

Specimen Type Temperature Time Special Container
Plasma Frozen 14 days

Specimen Minimum Volume

0.3 mL

Day(s) Performed

Monday through Friday

Specimen Retention Time

2 weeks

Report Available

3 to 5 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

Useful For

Follow-up of patients with maple syrup urine disease

 

Monitoring of dietary compliance for patients with maple syrup urine disease

CPT Code Information

82136

LOINC Code Information

Test ID Test Order Name Order LOINC Value
AAMSD Amino Acid, MSUD Panel, P 94566-7

 

Result ID Test Result Name Result LOINC Value
32446 Valine 94567-5
32447 Isoleucine 94568-3
32448 Leucine 94569-1
32449 Allo-isoleucine 94570-9
32450 Interpretation (AAMSD) 49247-0