Test Code LAB1231911 Amino Acids, Maple Syrup Urine Disease Panel, Plasma
Performing Laboratory
Mayo Clinic Laboratories in RochesterReporting Name
Amino Acid, MSUD Panel, PSpecimen Type
PlasmaOrdering 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 weeksReport Available
3 to 5 daysReference 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 |
Forms
If not ordering electronically, complete, print, and send a Biochemical Genetics Test Request (T798) with the specimen.