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Urine Coproporphyrin III

Immunology & Autoimmune

Coproporphyrin III/creatinine ratio (urine)U-COPRO-IIIUrinary Coproporphyrin IIIUrine coproporphyrin fraction III

Review status

Currently under review

Pending specialist review and validation.

What it shows

This test measures the amount of coproporphyrin III in your urine, adjusted to creatinine to account for how concentrated the urine is. Coproporphyrins are intermediate chemicals made during the production of heme, the molecule that carries oxygen in blood. Coproporphyrin III is one of the main isoforms and its level can reflect how well your body is making and clearing porphyrins.

Clinicians often order this test together with other porphyrin and porphobilinogen tests when evaluating symptoms that may suggest a porphyria or a problem with the liver or bile flow. Because many conditions can influence porphyrins, your result is interpreted in the context of other labs, your age, and your symptoms.

Why it matters

Abnormal urinary coproporphyrin III can be a clue to acute hepatic porphyrias, such as hereditary coproporphyria, and can also rise in several non-porphyria conditions like cholestasis, certain liver disorders, medication effects, and heavy metal exposure. It helps your clinician decide if further specialized porphyrin testing is needed, and whether your symptoms might relate to the heme pathway.

This test is often ordered when you have sudden severe abdominal pain, nausea, neurological symptoms, or dark urine, or when routine tests suggest impaired bile flow or liver injury. It can also support follow-up in known porphyria, helping monitor patterns over time alongside more specific tests.

Understanding your results

Results are considered together with your symptoms, other porphyrin measurements, and tests like urine porphobilinogen, fecal porphyrins, and sometimes plasma studies. A higher value may suggest increased production or reduced excretion of porphyrins, but it is not specific to one condition. Many common issues, including liver inflammation or certain medicines, can shift coproporphyrin patterns, so a single result rarely gives a final diagnosis.

If your result is higher than expected, your clinician may repeat the test with careful sample handling, review your medication and exposure history, and order additional fractionated porphyrin testing or genetic testing when appropriate. If your result is within expected limits yet your symptoms persist, testing during or soon after symptom flares may be advised. Discuss any concerns with your care team so follow-up is tailored to your situation.

Reference ranges

022.8 umol/mol creat.
All sexes
0 days – 7 days
033.1 umol/mol creat.
All sexes
7 days – 3 months
033.8 umol/mol creat.
All sexes
3 months – 6 months
052.6 umol/mol creat.
All sexes
6 months – 9 months
051.5 umol/mol creat.
All sexes
9 months – 12 months
048.8 umol/mol creat.
All sexes
12 months – 1 year
042.2 umol/mol creat.
All sexes
1 year – 2 years
032.7 umol/mol creat.
All sexes
2 years – 3 years
035.7 umol/mol creat.
All sexes
3 years – 4 years
033.4 umol/mol creat.
All sexes
4 years – 6 years
021.6 umol/mol creat.
All sexes
6 years – 9 years
023.9 umol/mol creat.
All sexes
9 years – 16 years
1.726.3 umol/mol 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 Coproporphyrin III

  • Sample handling and light exposure

    Porphyrins are light sensitive. Urine should be protected from light, promptly refrigerated or frozen, and tested quickly. Poor handling can degrade porphyrins and distort results.

  • Timing relative to symptoms

    Levels can change during symptom flares. Collecting urine at the onset of abdominal pain, nausea, or neurological symptoms can improve the test’s ability to detect clinically relevant changes.

  • Medications and substances

    Certain drugs and exposures, such as barbiturates, rifampin, sulfonamides, alcohol, and lead, can alter porphyrin metabolism or excretion. Always provide a full medication and exposure list.

  • Liver and bile flow conditions

    Cholestasis, hepatitis, and other hepatic disorders commonly shift coproporphyrin patterns. Interpreting results alongside liver enzymes and clinical context is essential.

  • Age and developmental stage

    Infants and young children can have different background porphyrin excretion patterns as metabolism matures. Age-specific interpretation helps avoid over-calling abnormalities.

  • Hydration and kidney function

    Results are normalized to creatinine, but extreme dehydration or significant kidney impairment can still affect interpretation. Let your clinician know about kidney issues.

2026

References

  1. McGill University Health Centre. (2014, October 28). U Coproporphyrin III (Task CD 11379912). Laboratory reference ranges.
  2. European Association for the Study of the Liver. (2017). EASL Clinical Practice Guidelines on the porphyrias. Journal of Hepatology, 66(2), 386–420.
  3. Balwani, M., & Desnick, R. J. (2019). The porphyrias: Advances in diagnosis and treatment. Blood, 133(20), 2185–2199.
  4. American Porphyria Foundation. (n.d.). Urine porphyrins: Testing and interpretation. External link