Related tests: protoporphyrin IX

Clinical significanceClinical indicationsSample typePreparationNotes

Clinical significance
Uroporphyrins are an intermediate product of the biosynthesis of haem, a structural component of haemoglobin. An increase is characteristic of the porphyrias, in which congenital or acquired enzyme deficiencies cause a block in the steps leading to the final synthesis of haem.

Clinical indications
Congenital and acquired porphyrias.

Sample type
Urine

Preparation
A urine sample must be collected.

Notes
For more complete information, testing for coproporphyrins and delta-aminolevulinic acid is useful.