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Urine Porphobilinogen

Urinalysis

PBGPorphobilinogen/creatinine ratio, urineUrinary porphobilinogen

Review status

Currently under review

Pending specialist review and validation.

What it shows

The urine porphobilinogen test measures porphobilinogen (PBG), a chemical made during the normal production of heme, which is the oxygen-carrying part of hemoglobin. When the heme pathway is disrupted, PBG can build up and spill into the urine. Because urine concentration can vary with hydration, many laboratories report PBG as a ratio to creatinine for a more stable comparison.

This test is often performed on a random urine sample, especially when symptoms are present. It is a key screening test for certain rare conditions called acute hepatic porphyrias, in which PBG typically rises during symptomatic episodes.

Why it matters

Clinicians order this test when you have symptoms that could suggest an acute porphyria attack, such as severe abdominal pain, nausea, constipation, limb pain or weakness, dark urine, or sudden mood and sleep changes. A timely result helps guide urgent management and can point toward inherited conditions affecting heme production.

Beyond diagnosis, the test may be used to track improvement after treatment or changes in triggers. When interpreted with your symptoms and other tests, it helps determine whether additional evaluations are needed, such as measuring related pathway chemicals, reviewing medications that may increase risk, or considering genetic testing.

Understanding your results

Your result is interpreted in the context of your symptoms and timing of collection. A result within the reference interval makes an acute porphyria attack less likely at that moment, although your clinician may repeat testing if symptoms are strongly suggestive or if the sample was not collected during a peak episode. If your result is higher than expected, your clinician may confirm with additional tests, assess for common triggers, and discuss treatment and preventive strategies.

If you are already receiving therapy or recently changed medications, results may shift and need careful interpretation. Kidney function, hydration, and sample handling can also influence findings. Always review your specific result with your clinician, who can advise on next steps, safety measures, and whether family testing or specialist referral is appropriate.

Reference ranges

-- umol/mmol Creat
All sexes
0 days – 3 years
00.9 umol/mmol Creat
All sexes
3 years – 7 years
00.6 umol/mmol Creat
All sexes
7 years – 12 years
00.6 umol/mmol Creat
All sexes
12 years – 16 years
02.36 umol/mmol Creat
All sexes
16 years – 150 years

Reference intervals vary by laboratory, analyzer, methodology, population, and units. The ranges shown here are for education only. Always interpret your results against the reference interval printed on your own lab report.

Factors that could impact Urine Porphobilinogen

  • Timing during symptoms

    PBG tends to rise during an acute attack and may approach usual levels between episodes. Testing during active symptoms is more informative than during quiet periods.

  • Sample handling and preservation

    PBG can degrade with heat or prolonged light exposure. Prompt refrigeration and timely transport improve accuracy. Follow collection instructions closely.

  • Hydration and urine concentration

    Very dilute or very concentrated urine alters raw concentrations. Reporting PBG relative to creatinine helps, but extreme dilution or concentration can still affect interpretation.

  • Medications and triggers

    Certain drugs, alcohol, smoking, infections, stress, and fasting or very low carbohydrate intake can increase pathway activity and influence PBG. Always share a full medication list.

  • Kidney function

    Reduced kidney function can change how PBG and creatinine are excreted, which may complicate the ratio and require clinical context for interpretation.

  • Pregnancy and hormonal changes

    Hormonal shifts, including pregnancy and the postpartum period, can affect attack risk. Discuss timing of symptoms and testing with your clinician if you are pregnant or planning pregnancy.

2026

References

  1. McGill University Health Centre. (2020, February 11). Urine Porphobilinogen (Task CD 4372404). Laboratory reference ranges.
  2. European Association for the Study of the Liver. (2023). EASL Clinical Practice Guidelines on porphyria. Journal of Hepatology.
  3. Balwani, M., & Desnick, R. J. (2019). The porphyrias: Advances in diagnosis and treatment. Blood, 133(23), 2525-2539.