Test Code UPGD Uroporphyrinogen Decarboxylase, Whole Blood
Performing Laboratory
Mayo Clinic Laboratories in RochesterReporting Name
UPG Decarboxylase, WBSpecimen Type
Whole bloodOrdering Guidance
Porphyria cutanea tarda (PCT) type I (sporadic), the most common form of PCT, exhibits normal erythrocyte enzyme activity. The preferred test for diagnosis of type I is PQNU / Porphyrins, Quantitative, 24 Hour, Urine or PQNRU / Porphyrins, Quantitative, Random, Urine.
Necessary Information
Include a list of medications the patient is currently taking.
Specimen Required
Patient Preparation: Patient must not consume any alcohol for 24 hours before specimen collection. This is essential as alcohol suppresses enzyme activity for 24 hours after ingestion.
Container/Tube:
Preferred: Green top (sodium heparin)
Acceptable: Lavender top (EDTA) or green top (lithium heparin)
Specimen Volume: 4 mL
Reject Due To
Gross hemolysis | Reject |
Specimen Stability Information
Specimen Type | Temperature | Time | Special Container |
---|---|---|---|
Whole blood | Refrigerated (preferred) | 14 days | |
Ambient | 7 days |
Specimen Minimum Volume
3 mL
Special Instructions
Day(s) Performed
Tuesday, Thursday
Specimen Retention Time
14 daysReport Available
2 to 6 daysReference Values
≥1.00 RU (normal)
0.80-0.99 RU (indeterminate)
<0.80 RU (indicative of PCT type II)
RU = Relative Units
Useful For
Preferred test for the confirmation of a diagnosis of porphyria cutanea tarda type II and hepatoerythropoietic porphyria
Testing Algorithm
The workup of patients with a suspected porphyria is most effective when following a stepwise approach. See Porphyria (Cutaneous) Testing Algorithm or call 800-533-1710 to discuss testing strategies. If guidance is needed for an acute form of porphyria, the Porphyria (Acute) Testing Algorithm is also available.
CPT Code Information
82657
LOINC Code Information
Test ID | Test Order Name | Order LOINC Value |
---|---|---|
UPGD | UPG Decarboxylase, WB | 49596-0 |
Result ID | Test Result Name | Result LOINC Value |
---|---|---|
8599 | UPG Decarboxylase, WB | 49596-0 |
606379 | Interpretation (UPGD) | 59462-2 |
606380 | Reviewed By | 18771-6 |
Genetics Test Information
Uroporphyrinogen decarboxylase is deficient in cases of type II porphyria cutanea tarda (PCT). Enzyme analysis is uninformative in sporadic PCT cases.
Forms
1. New York Clients-Informed consent is required. Document on the request form or electronic order that a copy is on file. The following documents are available:
-Informed Consent for Genetic Testing (T576)
-Informed Consent for Genetic Testing-Spanish (T826)
2. If not ordering electronically, complete, print, and send a Biochemical Genetics Test Request (T798) with the specimen.