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HelpOrder Code LABGOR, AAMSD Amino Acids, Maple Syrup Urine Disease Panel, Plasma
Additional Codes
Mayo Test Code: AAMSD
Reporting Name
Amino Acid, MSUD Panel, PUseful For
Follow-up of patients with maple syrup urine disease
Monitoring of dietary compliance for patients with maple syrup urine disease
Performing Laboratory
Mayo Clinic Laboratories in Rochester
Specimen 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.
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
Method Description
Quantitative analysis of amino acids is performed by liquid chromatography tandem mass spectrometry (LC-MS/MS). Patient samples are combined with isotopically labeled internal standard. Following protein precipitation, the supernatant is subjected to hydrophilic interaction liquid chromatography for the separation of isomers with MS/MS detection of the underivatized amino acids.(Unpublished Mayo method)
Reject Due To
| Gross hemolysis | OK |
| Gross lipemia | OK |
| Gross icterus | OK |
Method Name
Liquid Chromatography Tandem Mass Spectrometry (LC-MS/MS)
Portions of this test are covered by patents held by Quest Diagnostics
Forms
If not ordering electronically, complete, print, and send a Biochemical Genetics Test Request (T798) with the specimen.