Urinary Porphyrin Excretion in Various Types of Porphyria

Abstract

A new method of thin-layer chromatography was used for the study of urinary porphyrins in 42 porphyric patients (27 cases of porphyria cutanea tarda (PCT), 5 cases of porphyria variegata, 4 cases of acute intermittent porphyria, 2 cases of hereditary coproporphyria and 4 cases of erythropoietic protoporphyria), 21 of their clinically normal relatives and 5 controls. The results are compared to previously published data and discussed for the diagnosis of the porphyrias. If urinary porphyrin pattern seems sufficiently pathognomonic for PCT, it appears often unable to allow exact diagnosis of the acute porphyrias; faecal studies and sometimes enzymatic determinations are necessary.

Urinary Porphyrin Analyses in Patients With Porphyria Cutanea Tarda

“…This result may suggest that phlebotomy normalizes the uroporphyrinogen decarboxylase activity. Perrot et al (12) observed the disappearance of hepta -carboxyl porphyrin in the urine of a patient with porphyria cutanea tarda treated by phlebotomy.…”

“…Their method also produces excellent separation' but the procedure is a little difficult and cannot simultaneously analyze many samples. Perrot et al (12) described an important concept of urinary porphyrin analysis for the exact diagnosis of porphyrias. They reported one case of porphyria cutanea tarda with a low level of total urinary porphyrins.…”