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Test Code AAUCD Amino Acids, Urea Cycle Disorders Panel, Plasma

Reporting Name

Amino Acid, Urea Cycle Panel, P

Performing Laboratory

Mayo Clinic Laboratories in Rochester

Useful For

Follow-up of patients with urea cycle disorders

Method Name

Liquid Chromatography Tandem Mass Spectrometry (LC-MS/MS)


Ordering Guidance


Body fluids are not acceptable specimens for this test.

 

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

 

For testing on 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 the 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

Plasma

Specimen 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

Glutamine

<24 months: 356-857 nmol/mL

2-17 years: 353-790 nmol/mL

≥18 years: 447-774 nmol/mL

 

Ornithine

<24 months: 32-171 nmol/mL

2-17 years: 32-148 nmol/mL

≥18 years: 39-154 nmol/mL

 

Citrulline

<24 months: 8-42 nmol/mL

2-17 years: 12-44 nmol/mL

≥18 years: 18-57 nmol/mL

 

Arginine

<24 months: 28-164 nmol/mL

2-17 years: 28-156 nmol/mL

≥18 years: 45-144 nmol/mL

 

Argininosuccinic Acid

<5 nmol/mL

 

Reference value applies to all ages.

Interpretation

The quantitative results of glutamine, ornithine, citrulline, arginine, and argininosuccinic acid 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 days

Test 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
AAUCD Amino Acid, Urea Cycle Panel, P 100368-0

 

Result ID Test Result Name Result LOINC Value
32440 Glutamine 20643-3
32441 Citrulline 20640-9
32442 Argininosuccinic Acid 32227-1
32443 Arginine 20637-5
32444 Ornithine 20652-4
32445 Interpretation (AAUCD) 49247-0