What color is urine in porphyria patients? People with porphyria may have urine that is dark yellow to red or violet in color. How do you know if you need a urine porphyrin test?

Porphyria is diagnosed through biochemical analysis of blood, urine, and stool. [17] [31] In general, urine estimation of porphobilinogen (PBG) is the first step if acute porphyria is suspected.

Many patients have urine that is red/purple in color when they have an acute attack, but this is not always the case. Patients can still have elevated urine PBG levels even if their urine color is normal.

The isomer I porphyrins predominate in urine (as well as erythrocytes, plasma and feces) in Congenital Erythropoietic Porphyria (CEP), because of the marked deficiency of the enzyme UROS. Sodium carbonate (5 grams, added to a 24 hour urine bottle prior to collection) is widely recommended for urine specimens intended for measurement of PBG and ...

In the case of increased PBG in the urine sample, as the urine contacts the reagent, a cherry-red color develops immediately at the surface of the solution and then throughout the tube under agitation . The sensitivity of the test is typically 10 mg/L, while the upper normal limit for urinary PBG is approximately 2.5 mg/L.