Order Code PUPYU Purines and Pyrimidines Panel, Random, Urine
Useful For
Evaluating patients with symptoms suspicious for disorders of purine and pyrimidine metabolism
Monitoring patients with disorders of purine and pyrimidine metabolism
Laboratory evaluation of primary and secondary hyperuricemias
Method Name
Liquid Chromatography Tandem Mass Spectrometry (LC-MS/MS)
Reporting Name
Purines and Pyrimidines Panel, USpecimen Type
UrineOrdering Guidance
This is recommended screening test for the initial workup of a suspected disorder of purine and pyrimidine metabolism, particularly when clinical features are nonspecific, and includes measurement of purines, pyrimidines, uric acid, and S-sulfocysteine. If the clinical features are suggestive of molybdenum cofactor deficiency, isolated sulfite oxidase deficiency, and hereditary xanthinuria, order SSCTU / S-Sulfocysteine Panel, Urine.
If this test is ordered with SSCTU, then SSCTU will be canceled.
Necessary Information
Patient's age is required.
Specimen Required
Supplies: Urine Tubes, 10 mL (T068)
Container/Tube: Plastic, 10-mL urine tube
Specimen Volume: 3 mL
Collection Instructions: Collect a random urine specimen.
Specimen Minimum Volume
2 mL
Specimen Stability Information
Specimen Type | Temperature | Time | Special Container |
---|---|---|---|
Urine | Frozen | 90 days |
Reject Due To
All specimens will be evaluated at Mayo Clinic Laboratories for test suitability.Reference Values
Age range |
0-3 years |
4-6 years |
7-12 years |
13-18 years |
>18 years |
Uracil |
≤50 |
≤30 |
≤25 |
≤20 |
≤20 |
Thymine |
≤3 |
≤3 |
≤3 |
≤3 |
≤3 |
Adenine |
≤3 |
≤3 |
≤3 |
≤3 |
≤3 |
Hypoxanthine |
≤65 |
≤30 |
≤30 |
≤30 |
≤30 |
Xanthine |
≤54 |
≤21 |
≤35 |
≤15 |
≤20 |
Orotic |
≤4 |
≤4 |
≤3 |
≤3 |
≤5 |
Dihydroorotic acid |
≤3 |
≤3 |
≤3 |
≤3 |
≤3 |
Uric Acid |
350-2500 |
200-2000 |
200-1400 |
150-700 |
70-700 |
Deoxythymidine |
≤3 |
≤3 |
≤3 |
≤3 |
≤3 |
Deoxyuridine |
≤3 |
≤3 |
≤3 |
≤3 |
≤3 |
Thymidine |
≤3 |
≤3 |
≤3 |
≤3 |
≤3 |
Uridine |
≤10 |
≤3 |
≤3 |
≤3 |
≤3 |
Deoxyadenosine |
≤3 |
≤3 |
≤3 |
≤3 |
≤3 |
Deoxyinosine |
≤3 |
≤3 |
≤3 |
≤3 |
≤3 |
Deoxyguanosine |
≤3 |
≤3 |
≤3 |
≤3 |
≤3 |
Adenosine |
≤3 |
≤3 |
≤3 |
≤3 |
≤3 |
Inosine |
≤6 |
≤3 |
≤3 |
≤3 |
≤3 |
Guanosine |
≤4 |
≤3 |
≤3 |
≤3 |
≤3 |
5-Aminoimidazole-4-carboxamide 1-beta-D-ribofuranoside (AICAR) |
≤3 |
≤3 |
≤3 |
≤3 |
≤3 |
Succinyladenosine |
≤16 |
≤3 |
≤3 |
≤3 |
≤3 |
S-Sulfocysteine |
≤11 |
≤5 |
≤5 |
≤5 |
≤5 |
Dihydrouracil |
≤15 |
≤6 |
≤6 |
≤6 |
≤6 |
Dihydrothymine |
≤11 |
≤3 |
≤3 |
≤3 |
≤3 |
N-Carbamoyl-B-alanine |
≤30 |
≤10 |
≤10 |
≤10 |
≤10 |
N-Carbamoyl-B-aminoisobutyric |
≤20 |
≤3 |
≤3 |
≤3 |
≤3 |
All results reported as mmol/mol creatinine
Method Description
Diluted, filtered urine is mixed with an internal standard mixture and analyzed for uracil, thymine, adenine, hypoxanthine, xanthine, orotic, dihydroorotic, deoxythymidine, deoxyuridine, thymidine, uridine deoxyadenosine, deoxyinosine, deoxyguanosine, adenosine, inosine, guanosine, 5-aminoimidazole-4-carboxamide 1-beta-D-ribofuranoside, succinyladensoine, S-sulfocysteine, dihydrouracil, dihydrothymine, n-carbamoyl-beta-alanine, and N-carbamoyl-beta-aminoisobutyric acid by liquid chromatography-tandem mass spectrometry. The ratios of the extracted peak areas of the purine and pyrimidine analytes to the added internal standards are used to calculate the concentration of purines and pyrimidines present in the sample.(la Marca G, Casetta B, Malvagia S, et al. Implementing tandem mass spectrometry as a routine tool for characterizing the complete purine and pyrimidine metabolic profile in urine samples. J Mass Spectrom. 2006;41[11]:1442-1452; Monostori P, Klinke G, Hauke J, et al. Extended diagnosis of purine and pyrimidine disorders from urine: LC MS/MS assay development and clinical validation. PLoS One. 2019;14[2]:e0212458. doi:10.1371/journal.pone.0212458)
Day(s) Performed
Tuesday, Thursday
Performing Laboratory
Mayo Clinic Laboratories in RochesterCPT Code Information
82542
Forms
If not ordering electronically, complete, print, and send a Biochemical Genetics Test Request (T798) with the specimen.
Genetics Test Information
There are at least 35 known inherited disorders of purine and pyrimidine metabolism which cause a variety of neurological, immunological, hematological, and renal manifestations.