Porphobilinogen (PBG) Deaminase, RBC

Abbrev Code:ARMISC   
Order Code:LAB4909Order Name:Porphobilinogen Deaminase RBC
Synonyms:Erythrocyte Porphobilinogen (PBG) Deaminase
Methodology:Enzymatic fluorometry
CPT Codes: 82657 x1
Turnaround Time:Specimens are sent to reference laboratory Mon-Sat; results are reported within 1-6 days.
Special Instructions:Automated hemoglobin result must be included with specimen.

Collection Instructions

Specimen:Whole blood
Optimal Volume:3 mL
Minimum\Peds Volume:1 mL
Container:Purple (EDTA) on ICE
Collection Instructions:Perform automated hemoglobin on EDTA whole blood before preparing for transport.

Processing and Shipping

Specimen Processing:Do not centrifuge. Aliquot 3 mL, 1 mL minimum to an ARUP transport tube. Indicate specimen hemoglobin on container. Store whole blood frozen.
Shipping Instructions:Ship on dry ice.
Stability:Stability: 4 hours at room temperature; 1 week refrigerated; 1 month frozen.
Test Performed at or Referral Lab Lab Sendouts  (ARUP)
Referral Lab number:99550


Reference Range:2.10-4.30 mU/g Hb
Use:Porphobilinogen (PBG) deaminase (hydroxymethylbilane synthase or uroporphyrinogen I synthase) is expressed in units of mU per gram hemoglobin at 37 degrees C. In persons genetically susceptible to acute intermittent porphyria (AIP), PBG deaminase concentrations are approximately half of reference values. Normal concentrations of erythrocyte PBG deaminase may include abnormal forms of hepatic PBG deaminase.

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