UROPORPHYRINOGEN III SYNTHASE (CO-SYNTHASE) (UPG III S) ERYTHROCYTES

General Information

HLAB/HOL Code: UPS
UPHSM LIS Test #: 026950
Schedule:
Testing Time: 7-14 Days
Testing Lab: Mayo

Specimen Info
Only 1 specimen type required, unless otherwise specified

Volume: Full tube
Temperature: Refrigerate
Tube Type: Whole Blood (Heparin)
Collection Info:
ORDERING GUIDANCE
This test is most appropriately used for pediatric patients.

This test measures uroporphyrinogen (UPG) III synthase to confirm congenital erythropoietic porphyria, which is typically seen in early infancy. It does not measure UPG I synthase (also known as porphobilinogen deaminase), the enzyme deficient in acute intermittent porphyria (AIP). For AIP (and UPG I synthase), order PBGD_ / Porphobilinogen Deaminase, Whole Blood.

NECESSARY INFORMATION
1. Include a list of medications the patient is currently taking.
2. Date of transfusion, if performed

SPECIMEN REQUIRED
Patient Preparation: Patient should abstain from alcohol for 24 hours.
Container/Tube: Green top (heparin)
Specimen Volume: Full tube
Collection Instructions: Immediately place specimen on wet ice.

Specimen Acceptability

REJECT DUE TO:
Gross hemolysis: Reject

Methods

High-Performance Liquid Chromatography (HPLC)

Clinical Utilities

Diagnosis of congenital erythropoietic porphyria
This test is not useful for diagnosis of acute intermittent porphyria (AIP).

CPT Codes

82657

* The CPT codes provided are based on AMA guidelines and are for informational purposes only. CPT coding
is the sole responsibility of the billing party. Please direct any questions regarding coding to the payer being billed.