ポルフィリン症患者およびその未発症血縁者における酸化的ストレスに関する研究

Abstract

We sought to establish a causal relationship between oxidative stress and porphyria in patients and carriers. We reported changes in urinary porphyrin concentrations related to 8-hydroxy-2'-deoxyguanosine. We measured urinary 8-hydroxy-2'-deoxyguanosine concentration in porphyria patients and carriers with multifactorial inheritance as a possible marker of attack. The porphyria types included 10 patients with porphyria cutanea tarda, 5 with variegate porphyria, 8 with hereditary coproporphyria, 7 with congenital erythropoietic porphyria, 5 with erythropoietic protoporphyria, 5 with acute intermittent porphyria, 7 erythropoietic protoporphyria carriers, and 7 acute intermittent porphyria carriers. Urinary porphyrin concentrations in these patients were significantly higher than those in healthy subjects (p<0.001). Urinary 8-hydroxy-2'-deoxyguanosine concentrations were significantly high in dermatopathy porphyria types namely porphyria cutanea tarda (p<0.001), variegate porphyria (p<0.05), hereditary coproporphyria (p<0.05), congenital erythropoietic phyria (p<0.05), and erythropoietic protoporphyria (p<0.001). These results reveal that urinary 8-hydroxy-2'-deoxyguanosine concentration in cutis porphyria types is a good predictor of attack and abatement.