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80288M
UROPORPHYRINOGEN III SYNTHASE (CO-SYNTHASE) (UPG III S), ERYTHROCYTES

Test Code 80288M 
Test UROPORPHYRINOGEN III SYNTHASE (CO-SYNTHASE) (Upg III S), ERYTHROCYTES 
Methodology High Performance Liquid Chromatography (HPLC)
Note: This test measures UPG III synthase to diagnose
congenital erythropoietic porphyria. It does not
measure UPG I synthase, the enzyme deficiency
responsible for acute intermittent porphyria (AIP).
For AIP (and UPG I synthase), order #88925
"Porphobilinogen (PBG) Deaminase, Erythrocytes."
See "Porphyria (Acute) Testing Algorithm" and
"Porphyria (Cutaneous) Testing Algorithm" in
Special Instructions for additional information. 
Description Useful for investigation of the feasibility of CEP and as the basis for biochemical diagnosis of CEP 
Specimen All porphyrin tests on erythrocytes can be performed on 1 draw
tube.

Specimen must arrive within 48 hours of draw.

1. Patient should abstain from alcohol for 24 hours.
Note: Include a list of medications the patient is currently taking and forward with the specimen.
2. Draw a full, green-top (heparin) tube and place on wet ice
immediately.
3. Send entire heparinized whole blood specimen (minimum 3 mL whole blood) refrigerated.
Specimen cannot be frozen.
4. Test will be cancelled if frozen whole blood is received. 

Reference Values >= 75 Relative Units (normal)

See "Porphyrinogens, Porphyrins, and Porphyrias." 

Analytic Time 7 Days 
Day(s) Test Set Up Varies 
CPT Code(s) 82657  

Special Instructions