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ID:332 Porphobilinogen (PBG)
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DescriptionPorphobilinogen (PBG) is a pyrrole involved in porphyrin metabolism. It is generated by aminolevulinate (ALA) and the enzyme ALA dehydratase. PBG is then converted into hydroxymethyl bilane by the enzyme porphobilinogen deaminase.
IndicationInvestigation of suspected acute porphyria and acute abdomen of unknown cause.
Additional InfoFor acute porphyrias collect samples when patients are symptomatic. Contact Dr JH Barth (via switchboard) or Dr M Henderson/Tim Degg ext 66861 or x66063. If urgent analysis is required please contact the duty biochemist or on-call duty consultant (via switchboard).
Concurrent Testsna
Dietary Requirementsna
InterpretationAn increased PBG excretion is diagnostic of acute porphyria. The specific diagnosis will require further tests (see additional info).

DepartmentSpecialist Laboratory Medicine
SampleUrine
TubePlain Universal
Tube Picture
Collection ConditionsPlain random urine. Protect from light. Envelope or black polythene/silver foil will do. Do not discard if not protected from light just comment on form.
Min. Vol10 mL
Freq.Weekly

Ref. Range (Male) <0.9 umol/mmol creat
Ref. Range (Female)<0.9 umol/mmol creat
Ref. Range (Paed) <0.9 umol/mmol creat
Ref. Range Notes
Unitsumol/mmol (creat)
IP Acute TAT- Contact Laboratory
IP Routine TAT14 days
GP Acute TAT- Contact Laboratory
GP Routine TAT14 days
Turnround CommentNA

Originally edited by : . Review due on 04/07/2019 08:34:07. Published By RB on 04/07/2018 08:34:07.