{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2024,2,14]],"date-time":"2024-02-14T21:40:07Z","timestamp":1707946807584},"reference-count":46,"publisher":"Akademiai Kiado Zrt.","issue":"23","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2007,6,1]]},"abstract":"<jats:p>Az elm\u00falt \u00e9vtizedekben egyre t\u00f6bb betegs\u00e9gr\u0151l der\u00fclt ki, hogy patogenezis\u00e9ben az oxidat\u00edv stressznek \u00e9s a f\u00e9mionoknak fontos szerep\u00fck van. Ez a magyar\u00e1zata annak, hogy az antioxid\u00e1ns-kezel\u00e9s jelent\u0151s\u00e9ge fel\u00e9rt\u00e9kel\u0151d\u00f6tt, \u00e9s napjaink kutat\u00e1saiban is el\u0151t\u00e9rbe ker\u00fclt. Porphyria cutanea tard\u00e1ban (PCT) a hem bioszint\u00e9zise k\u00e1rosodik, amely az uroporfirinog\u00e9n-dekarboxil\u00e1z enzim cs\u00f6kkent aktivit\u00e1s\u00e1nak k\u00f6vetkezt\u00e9ben alakul ki. A hem-prekurzorok akkumul\u00e1l\u00f3dnak a v\u00e9rben, a m\u00e1jban \u00e9s a b\u0151rben. A hem-prekurzorok \u00e9s a porfirin a sz\u00e9klettel \u00e9s a vizelettel \u00fcr\u00fclnek. Az enzimdefektus autoszom\u00e1lis domin\u00e1ns. A b\u0151rt\u00fcnetek napf\u00e9ny hat\u00e1s\u00e1ra feler\u0151s\u00f6dnek. Ennek oka a b\u0151rben felszaporod\u00f3 uroporfirinek \u00e9s a heptakarboxilporfirin f\u00e9ny\u00e9rz\u00e9keny\u00edt\u0151, valamint a vas lipidperoxid\u00e1ci\u00f3t kiv\u00e1lt\u00f3 hat\u00e1sa. A genetikai eredet mellett az alkoholfogyaszt\u00e1s, hepatotoxikus vegy\u00fcletek, \u00f6sztrog\u00e9n \u00e9s a v\u00edrusfert\u0151z\u00e9sek is hozz\u00e1j\u00e1rulnak a betegs\u00e9g kialakul\u00e1s\u00e1hoz. Az elfogadott kezel\u00e9si m\u00f3d az id\u0151nk\u00e9nti v\u00e9rlebocs\u00e1t\u00e1s. A m\u00e1jk\u00e1rosod\u00e1s talaj\u00e1n kialakul\u00f3, sporadikus porphyria cutanea tarda eset\u00e9ben a felhalmoz\u00f3d\u00f3 vas felel\u0151s els\u0151sorban az oxidat\u00edv stressz kialakul\u00e1s\u00e1\u00e9rt. Megv\u00e1ltozik a betegek redox-homeostasisa \u00e9s az antioxid\u00e1nsszint cs\u00f6kken. A m\u00e1j redoxst\u00e1tusz\u00e1nak vizsg\u00e1lata, valamint az antioxid\u00e1ns kieg\u00e9sz\u00edt\u0151 kezel\u00e9s hat\u00e1s\u00e1nak k\u00f6vet\u00e9se phlebotomiz\u00e1lt porphyria cutanea tard\u00e1ban szenved\u0151 betegekn\u00e9l a klinikai diagnosztika mellett biok\u00e9miai \u00e9s f\u00e9manalitikai m\u00f3dszerekkel t\u00f6rt\u00e9nhet. Vizsg\u00e1latok igazolt\u00e1k, hogy a phlebotomia j\u00f3 kezel\u00e9si m\u00f3dszernek bizonyult a phototoxikus b\u0151rt\u00fcnetek kezel\u00e9s\u00e9ben, de az elj\u00e1r\u00e1s nem v\u00e1ltoztatta meg kedvez\u0151en a f\u00e9mionok egym\u00e1shoz viszony\u00edtott ar\u00e1ny\u00e1t a betegek v\u00e9r\u00e9ben.<\/jats:p>","DOI":"10.1556\/oh.2007.28103","type":"journal-article","created":{"date-parts":[[2007,6,5]],"date-time":"2007-06-05T20:57:35Z","timestamp":1181077055000},"page":"1095-1100","source":"Crossref","is-referenced-by-count":1,"title":["Importance of metal ion metabolism and oxidative stress in male patients suffered with porphyria cutanea tarda"],"prefix":"10.1556","volume":"148","author":[{"given":"Edit","family":"Sz\u00e9kely","sequence":"first","affiliation":[{"name":"1 M\u00c1V K\u00f3rh\u00e1z \u00e9s Rendel\u0151int\u00e9zet Orsz\u00e1gos Porphyria K\u00f6zpont Budapest Podmaniczky u. 111. 1062"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Gy\u00f6ngyi","family":"Tasn\u00e1di","sequence":"additional","affiliation":[{"name":"1 M\u00c1V K\u00f3rh\u00e1z \u00e9s Rendel\u0151int\u00e9zet Orsz\u00e1gos Porphyria K\u00f6zpont Budapest Podmaniczky u. 111. 1062"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Kl\u00e1ra","family":"Szentmih\u00e1lyi","sequence":"additional","affiliation":[{"name":"2 Magyar Tudom\u00e1nyos Akad\u00e9mia, K\u00e9miai Kutat\u00f3k\u00f6zpont Budapest"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Andrea","family":"Alm\u00e1si","sequence":"additional","affiliation":[{"name":"1 M\u00c1V K\u00f3rh\u00e1z \u00e9s Rendel\u0151int\u00e9zet Orsz\u00e1gos Porphyria K\u00f6zpont Budapest Podmaniczky u. 111. 1062"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"M\u00e1rta","family":"Bor","sequence":"additional","affiliation":[{"name":"1 M\u00c1V K\u00f3rh\u00e1z \u00e9s Rendel\u0151int\u00e9zet Orsz\u00e1gos Porphyria K\u00f6zpont Budapest Podmaniczky u. 111. 1062"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"J\u00e1nos","family":"Feh\u00e9r","sequence":"additional","affiliation":[{"name":"3 Semmelweis Egyetem, \u00c1ltal\u00e1nos Orvostudom\u00e1nyi Kar II. 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Dermatol."},{"key":"atypb6","volume-title":"Seminars in dermatology","year":"1986"},{"key":"atypb7","doi-asserted-by":"crossref","first-page":"181","DOI":"10.1002\/hep.510270128","volume":"27","year":"1998","journal-title":"Hepatology"},{"key":"atypb8","doi-asserted-by":"crossref","first-page":"321","DOI":"10.1016\/S0140-6736(96)09436-6","volume":"349","year":"1997","journal-title":"Lancet"},{"key":"atypb9","first-page":"33","volume":"48","year":"2002","journal-title":"Cell. Mol. Biol."},{"key":"atypb10","first-page":"17","volume":"32","year":"2002","journal-title":"Clin. Chim. Acta"},{"key":"atypb11","doi-asserted-by":"crossref","first-page":"S22","DOI":"10.1038\/sj.ki.4490847","volume":"55","year":"1999","journal-title":"Kidney Int."},{"key":"atypb12","first-page":"155","volume":"78","year":"1993","journal-title":"Nebraska Med. J."},{"key":"atypb13","first-page":"1089","volume":"50","year":"2000","journal-title":"Rev. Prat."},{"key":"atypb14","first-page":"183","volume":"63","year":"2003","journal-title":"Med. Trop."},{"key":"atypb15","volume-title":"Disorders of porphyrin metabolism","year":"1987"},{"key":"atypb16","first-page":"1351","volume":"153","year":"1967","journal-title":"Derm. Wochenschr."},{"key":"atypb17","doi-asserted-by":"crossref","first-page":"805","DOI":"10.1053\/jhep.2002.35621","volume":"36","year":"2002","journal-title":"Hepatol."},{"key":"atypb18","doi-asserted-by":"crossref","first-page":"482","DOI":"10.1067\/mlc.2000.111024","volume":"136","year":"2000","journal-title":"J. Lab. Clin. Med."},{"key":"atypb19","doi-asserted-by":"crossref","first-page":"30","DOI":"10.1007\/s11894-999-0084-5","volume":"1","year":"1999","journal-title":"Curr. Gastroenterol. Rep."},{"key":"atypb20","doi-asserted-by":"crossref","first-page":"561","DOI":"10.1080\/003655100448338","volume":"60","year":"2000","journal-title":"Scand. J. Clin. Lab. Invest."},{"key":"atypb21","doi-asserted-by":"crossref","first-page":"241","DOI":"10.1016\/S0140-6736(05)70154-9","volume":"365","year":"2005","journal-title":"Lancet"},{"key":"atypb22","doi-asserted-by":"crossref","first-page":"1565","DOI":"10.1182\/blood.V95.5.1565.005k42_1565_1571","volume":"95","year":"2000","journal-title":"Blood"},{"key":"atypb23","doi-asserted-by":"crossref","first-page":"398","DOI":"10.1111\/1523-1747.ep12363384","volume":"107","year":"1996","journal-title":"J. Invest. Dermatol."},{"key":"atypb24","doi-asserted-by":"crossref","first-page":"177","DOI":"10.1016\/0003-9861(90)90429-3","volume":"281","year":"1990","journal-title":"Arch. Biochem. Biophysics"},{"key":"atypb25","doi-asserted-by":"crossref","first-page":"865","DOI":"10.3109\/00498259009046902","volume":"20","year":"1990","journal-title":"Xenobiotica"},{"key":"atypb26","doi-asserted-by":"crossref","first-page":"97","DOI":"10.1016\/S0378-4274(01)00350-2","volume":"122","year":"2001","journal-title":"Toxicology Lett."},{"key":"atypb27","doi-asserted-by":"crossref","first-page":"25","DOI":"10.1007\/s00204-003-0470-y","volume":"78","year":"2004","journal-title":"Arch. Toxicol."},{"key":"atypb28","doi-asserted-by":"crossref","first-page":"123","DOI":"10.1007\/s00592-005-0191-1","volume":"42","year":"2005","journal-title":"Acta Diabetol."},{"key":"atypb29","doi-asserted-by":"crossref","first-page":"457","DOI":"10.1016\/S0891-5849(02)00842-0","volume":"33","year":"2002","journal-title":"Free Radic. Biol., Med."},{"key":"atypb30","first-page":"61","volume":"56","year":"2003","journal-title":"Wiad. Lek."},{"key":"atypb31","doi-asserted-by":"crossref","first-page":"1928","DOI":"10.1097\/01.alc.0000187595.19324.ca","volume":"29","year":"2005","journal-title":"Alc. Clin. Exp. Res."},{"key":"atypb32","doi-asserted-by":"crossref","first-page":"314","DOI":"10.1007\/BF03044890","volume":"94","year":"1999","journal-title":"Medizinische Klinik."},{"key":"atypb33","doi-asserted-by":"crossref","first-page":"1998","DOI":"10.1002\/ijc.21583","volume":"118","year":"2006","journal-title":"Int. J. Cancer."},{"key":"atypb34","first-page":"1","volume":"2","year":"2000","journal-title":"Eur. J. Gastroent. Hepatol."},{"key":"atypb35","doi-asserted-by":"crossref","first-page":"567","DOI":"10.1007\/s10534-005-8488-7","volume":"18","year":"2005","journal-title":"Biometals"},{"key":"atypb36","doi-asserted-by":"crossref","first-page":"349","DOI":"10.1016\/0014-5793(89)80753-7","volume":"250","year":"1989","journal-title":"FEBS Lett."},{"key":"atypb37","doi-asserted-by":"crossref","first-page":"1324","DOI":"10.1016\/0006-291X(87)90794-7","volume":"146","year":"1987","journal-title":"Biochem. Biophys. Res. Commun."},{"key":"atypb38","first-page":"1","volume":"35","year":"1998","journal-title":"Metal Ions Biol. Syst."},{"key":"atypb39","first-page":"1853","volume":"145","year":"2004","journal-title":"Orv. Hetil."},{"key":"atypb40","first-page":"515","volume":"43","year":"2005","journal-title":"Z. Gastronenterol."},{"key":"atypb41","first-page":"1882","volume":"26","year":"2005","journal-title":"Saudi Med. J."},{"key":"atypb42","doi-asserted-by":"crossref","first-page":"240","DOI":"10.5414\/TEP21240","volume":"21","year":"2004","journal-title":"Trace Elem. Electrol."},{"key":"atypb43","doi-asserted-by":"crossref","first-page":"75","DOI":"10.1080\/10715760500312305","volume":"40","year":"2006","journal-title":"Free Radic Res."},{"key":"atypb44","doi-asserted-by":"crossref","first-page":"102","DOI":"10.1016\/S0891-5849(97)00169-X","volume":"24","year":"1998","journal-title":"Free Rad. Biol. Med."},{"key":"atypb45","doi-asserted-by":"crossref","first-page":"217","DOI":"10.1016\/j.jtemb.2005.07.011","volume":"19","year":"2005","journal-title":"Trace Elem. Med. Biol."},{"key":"atypb46","first-page":"403","volume":"42","year":"2004","journal-title":"Z. Gastroenterol."},{"key":"atypb47","first-page":"43","volume":"1","year":"2006","journal-title":"Trace Elem. 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