Urine Porphobilinogen and Aminolevulinic Acid
- Description
- Urine porphobilinogen (PBG) and 5-aminolevullinic acid (ALA) is a test profile offered by the SAS Porphyria Service in Reference Biochemisty at the Synnovis hub. This test is indicated in the diagnosis and monitoring of patients with acute porphyria. Quantitative measurement is by ion-exchange chromatography followed by spectrophotometry.
- Clinical details
ALA and PBG are precursors in the haem biosynthesis pathway and are significantly elevated in patients during an acute porphyria attack. Levels can remain elevated for weeks following an attack, or, in the case of acute intermittent porphyria (AIP), years. Moderate increases in ALA excretion can also be seen in patients with hereditary tyrosinaemia and lead toxicity. Urine PBG and ALA are analysed and reported together and reported as excretion per mmol of creatinine. Urgent PBG analysis is available during working hours (sample must arrive in lab by 3pm) and can be arranged by prior agreement with both the porphyria laboratory (contact details below) and the National Acute Porphyria Service (NAPS) on 029 2074 7747
- Related condition
- Reference range
PBG: 0.0 – 1.5 umol/mmol creatinine
ALA: 0.0 – 3.8 umol/mmol creatinine
- Units
umol/mmol creatinine
- Synonyms
ALA, PBG, porphobilinogen, aminolevullinic acid
- Testing site
- Synnovis : Reference Services : Blackfriars Hub
- Laboratory
- Porphyrins
- Sample type and volume required
10-20 mL fresh urine, preferably collected early morning. No preservative is necessary (plain unviversl)
- Special sample instructions
Protect from light
- Storage and transport
Store refrigerated. Send overnight at room temperature. Protect samples from light. If delay in dispatch, store and send frozen.
- Turnaround time
Routine: 7-14 days Urgent PBG: Same day provided sample received by 3pm, otherwise next working day
- Contacts
Porphyrin Laboratory at Blackfriars Hub
Phone: 0208 078 8996
Email: PorphyrinLab@synnovis.co.uk
Porphyrin Laboratory
Floor 1, Synnovis hub
Friars Bridge Court
41-43 Blackfriars Road
London, SE1 8NZ