Home
HelpTest Code AAMSD Amino Acids, Maple Syrup Urine Disease Panel, Plasma
Reporting Name
Amino Acid, MSUD Panel, PPerforming Laboratory
Mayo Clinic Laboratories in Rochester
Useful For
Follow-up of patients with maple syrup urine disease
Monitoring of dietary compliance for patients with maple syrup urine disease
Method Name
Liquid Chromatography Tandem Mass Spectrometry (LC-MS/MS)
Portions of this test are covered by patents held by Quest Diagnostics
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 Type
PlasmaSpecimen Minimum Volume
0.3 mL
Specimen Stability Information
| Specimen Type | Temperature | Time | Special Container |
|---|---|---|---|
| Plasma | Frozen | 14 days |
Reject Due To
| Gross hemolysis | OK |
| Gross lipemia | OK |
| Gross icterus | OK |
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
Interpretation
The quantitative results of isoleucine, leucine, valine, and allo-isoleucine with age-dependent reference values are reported without added interpretation. When applicable, reports of abnormal results may contain an interpretation based on available clinical interpretation.
Day(s) Performed
Monday through Friday
Report Available
3 to 5 daysTest Classification
This test was developed and its performance characteristics determined by Mayo Clinic in a manner consistent with CLIA requirements. It has not been cleared or approved by the US Food and Drug Administration.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.