[Significance of metal ion metabolism and oxidative stress in male patients with porphyria cutanea tarda].
In the last decades, the role of oxidative stress and trace elements was proven to play an important role in the pathogenesis of more and more diseases. This is why a great importance is attributed lately to the antioxidant therapy, and lots of studies are dealing with this issue. In porphyria cutanea tarda (PCT) the biosynthesis of hem is damaged, because of the reduced activity of uroporphyrinogen-decarboxylase enzyme. The hem precursors are accumulating in blood, liver and skin. The hem precursors and porphyrin are eliminated with urine and stool. The enzyme defect is autosomal dominant. The skin symptoms are intensified by sun exposure. This is because the accumulation of uroporphyrins and heptacarboxylporphyrin in the skin causes photosensitivity, and the accumulated iron has a lipid-peroxidation effect. Besides the genetical origin, the alcohol consumption, the hepatotoxic drugs, estrogen and viral infections can also determine the development of the disease. The applied treatment is phlebotomy. In the case of PCT that appears in the field of liver damage, the accumulation of iron is responsible for the development of oxidative stress. The patient's redox homeostasis is changed, and the level of antioxidants is decreased. The redox state of liver and the effects of additional antioxidant treatment in phlebotomized PCT patients were determined by biochemical and trace element analytical methods. According to the clinical data, phlebotomy proved to be an effective treatment in PCT patients. Phlebotomy improved the phototoxic skin symptoms, but it did not improve the ratios of trace elements to each other in the blood of the patients.
Székely
Edit
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MAV Kórház és Rendelointézet Országos Porphyria Központ Budapest Podmaniczky u. 111. 1062, Hungary. szekedit@freemail.hu
Tasnádi
Gyöngyi
G
Szentmihályi
Klára
K
Almási
Andrea
A
Bor
Márta
M
Fehér
János
J
Blázovics
Anna
A
D000328:Adult; D000368:Aged; D006801:Humans; D007501:Iron / Q000378:metabolism*; D019190:Iron Overload / Q000378:metabolism*; D008099:Liver / Q000378:metabolism*; D008108:Liver Diseases, Alcoholic / Q000378:metabolism; D008297:Male; D008875:Middle Aged; D010084:Oxidation-Reduction; D018384:Oxidative Stress; D018962:Phlebotomy; D017119:Porphyria Cutanea Tarda / Q000235:genetics / Q000378:metabolism* / Q000628:therapy; D011166:Porphyrins / Q000378:metabolism; D014131:Trace Elements / Q000378:metabolism*; D014575:Uroporphyrinogen Decarboxylase / Q000235:genetics / Q000378:metabolism
D011166:Porphyrins; D014131:Trace Elements; D007501:Iron; D014575:Uroporphyrinogen Decarboxylase