His urinary porphyrin excretion profile showed greatly elevated porphyrin concentrations (10- to 13-fold) with a preponderance of uroporphyrin
, typical of acute porphyria.
Porphobilinogen was found to be strongly positive in the analysis of a spot urine sample, and her uroporphyrin
, corpoporphyrin and total porphyrin levels were found to be elevated in a 24-hour urine collection.
6) Abnormal porphyrin metabolism, a characteristic of diseases usually inherited as an enzyme deficiency and resulting in abnormal synthesis of photodynamic agents, including uroporphyrin
and coproporphyrin, can result in photosensitization.
Differential potency of atropisomers of polychlorinated biphenyls on cytochrome P450 induction and uroporphyrin
accumulation in the chick embryo hepatocyte culture.
The steps in the heme synthesis pathway that are most vulnerable to heavy metal inhibition are those that involve the enzymes uroporphyrin
decarboxylase (UROD) and coproporphyrinogen oxidase (CPOX).
Quantum yields and kinetics of the photobleaching of hematoporphyrin, photofrin II, tetra(4-sulfonatophenyl)-porphine and uroporphyrin
CYP3A-inducing agents and the attenuation of uroporphyrin
accumulation and excretion in a rat model of porphyria cutanea tarda.
PbAs had additive effects on urinary excretion of coproporphyrin, but less than additive for uroporphyrin
Analysis has suggested that this fluorescence results from a mixture of porphyrins, including a mixture of uroporphyrin
, harderoporphyrin, isoharderoporphyrin, protoporphyrin, and pentacarboxylic porphyrin loosely bound to proteins, coupled with a second group that may be derivatives of a dicarboxylic porphyrin tightly bound to albumin (14, 17).
For example, in erythropoietic protoporphyria (EPP) there is an excess of protoporphyrin (PP) while in congenital erythropoietic porphyria (Gunther's disease) there are high levels of uroporphyrin
(UP), the photosensitivity being more severe in this case (26).
In 66 cases (35 symptomatic and 31 symptom free), the diagnosis was based on low erythrocyte PBGD activity (<55 [micro]mol uroporphyrin
Key words: coproporphyrin I, coproporphyrin III, hexachlorobenzene, neonates, porphyria, uroporphyrin