The porphyrias: a review.

Porphyria cutanea tarda-like Syndrome in Hemodialyzed Patients

“…An analysis of the aluminium content of the patients' skin or liver has not been performed. Porphyria cutanea tarda is frequently as sociated with chronic liver diseases, particu larly those due to alcohol excess or to dis turbances in iron metabolism, and with estrogen therapy; furthermore, the majority of patients are men between 40 and 60 [16]. 2 of our patients had hepatic hemosiderosis associ ated with massive transfusion.…”

“…On the other hand, alterations in fecal por phyrin concentration are less striking in these established syndromes [16]. The hypothesis of a toxic drug reaction secondary to photo sensitization of sun-exposed areas is improb able because of the clinical aspects and the histological findings which should include significant lymphoplasmocellular infiltration and cell necrosis.…”

The Photochemistry of Haematoporphyrin and Related Systems

“…The porphyrins which occur in the porphyrias arise by a dysfunction of haem biosynthesis and vary in structure depending on the particular step in the biosynthetic pathway at which the enzymic dysfunction occurs. Thus, for example, in erythropoietic protoporphyria (EPP) there is an excess of protoporphyrin (PP) while in congenital erythropoietic porphyria (Ginther's disease) there are high levels of uroporphyrin (UP), the photosensitivity being more severe in this case (Elder et al 1972). Photohaemolysis work supports the idea of different porphyrins varying in their effectiveness as photosensitizers so that it has been of interest to examine the photophysical properties of the porphyrins concerned.…”

The Effect of the Porphyrogenic Compound, Hexachlorobenzene, on the Activity of Hepatic Uroporphyrinogen Decarboxylase in the Rat

“…HCBC') poisoning was responsible for an outbreak of porphyria in Turkey in 1956 (Schmid, 1960) and exposure to other polychlorinated aromatic hydrocarbons may cause porphyria in workers manufacturing herbicides (Bleiberg, Wallen, Brodkin & Applebaum, 1974) or handling disinfectants (Lynch, Lee & Kushner, 1975). In man, these compounds are one cause of the syndrome known as porphyria cutanea tarda symptomatica or symptomatic porphyria, a form of hepatic porphyria which is seen most commonly in association with liver damage due to alcohol (Elder, Gray & Nicholson, 1972). Iron stores are increased in about two-thirds of European patients with symptomatic porphyria (Lundvall, 1971 ;Turnbull, Baker, Vernon-Roberts & Magnus, 1973).…”