The Leeds Teaching Hospitals NHS Trust

Porphyrins (urine)

Porphyrins (urine)

The acute porphyrias include AIP, HCP, VP and may present with the following symptoms: abdominal pain, psychiatric disorders, peripheral neuropathy. 75% of attacks are precipiated by either drugs (including oral contraceptives) or alcohol. With the exception of AIP, all the above can be associated with dermatological problems. Lab screen for AIP: measure urine PBG. If negative, provided sample has been collected during an acute attack, AIP is unlikely, though not impossible. Clinical indication for request: all types of acute porphyria and all types of cutaneous porphyria. Patients who present with dermatological involvement require full assesment: urine, faeces and blood should be analysed.

Tube 08 (UR) - Urine
ID Tests&Tubes0235
Additional Information If acute porphyria is suspected, collect samples when patient is symptomatic. Contact Duty Biochemist (0113 39 26922 option 2) or National Acute Porphyria Service (029 2074 7747). If urgent analysis is required please contact the duty biochemist (26922 Opt 2) or on-call duty consultant (via switchboard). Please also send 2x 5mL EDTA blood samples (purple top) for blood porphyrin analysis. Measurement of urinary PBG alone is used as the screening for acute porphyria (test may be unreliable if creatinine < 4 mmol/L).
Turn Around Turnaround time stated by Cardiff porphyria service: 10 working days
Send to Blood Sciences LGI
(Test referred to: Cardiff porphyria service.)
Contact For further details please contact Leeds Pathology customer services: leedsth-tr.pathologycustomerservice@nhs.net.
Collection Con Early morning urine sample preferred. Please also send 2x EDTA blood tubes. PROTECT ALL SAMPLES FROM LIGHT. Instructions to lab: store in fridge until dispatch. Note on telepath (specimen comment) whether sample was light-protected. Samples that have not been protected from light are ok to send to Cardiff if < 6h. Samples exposed to light for > 6h should not be sent.
Sample REQ Urine
Ref. Range Notes urine porphyrin/creatinine ratio < 40 nmol/mmol creatinine. Urine porphobilinogen < 10.7 umol/L
Telepath LGI superset LPORUR. SJUH superset JPORUR.