{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,3,31]],"date-time":"2025-03-31T16:27:31Z","timestamp":1743438451167},"reference-count":22,"publisher":"Akademiai Kiado Zrt.","issue":"29","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2010,7,1]]},"abstract":"<jats:p>Az amyloidosis k\u00fcl\u00f6nb\u00f6z\u0151 eredet\u0171 fibrill\u00e1ris jelleg\u0171 anyag extracellul\u00e1ris felhalmoz\u00f3d\u00e1sa. K\u00fcl\u00f6nb\u00f6z\u0151 szervekben lerak\u00f3dva azok m\u0171k\u00f6d\u00e9s\u00e9t k\u00e1ros\u00edtja. A funkci\u00f3k\u00e1rosod\u00e1sb\u00f3l ad\u00f3dnak a klinikai t\u00fcnetek, az agyon k\u00edv\u00fcl minden szerv \u00e9rintett lehet, leggyakrabban a vese, a m\u00e1j, a l\u00e9p, a gastrointestinalis rendszer, az endokrin szervek, a b\u0151r, a sz\u00edv, a t\u00fcd\u0151 \u00e9s a vegetat\u00edv idegrendszer. Speci\u00e1lis sz\u00f6vettani m\u00f3dszerek (kong\u00f3v\u00f6r\u00f6s fest\u00e9s, polariz\u00e1lt f\u00e9nyben val\u00f3 \u00e9s elektronmikroszk\u00f3pos, valamint immunohisztol\u00f3giai vizsg\u00e1latok), adott klinikum eset\u00e9n genetikai tesztek sz\u00fcks\u00e9gesek a diagn\u00f3zishoz. Az izot\u00f3ppal jelzett sz\u00e9rum-amyloid-P-komponenssel v\u00e9gzett szcintigr\u00e1fia a betegs\u00e9g kiterjed\u00e9s\u00e9t, pontos lokaliz\u00e1ci\u00f3j\u00e1t mutatja meg, valamint seg\u00edt a kezel\u00e9s hat\u00e1s\u00e1nak meg\u00edt\u00e9l\u00e9s\u00e9ben. Leggyakoribb a plazmasejt-betegs\u00e9gekhez t\u00e1rsul\u00f3, k\u00f6nny\u0171l\u00e1nc-eredet\u0171 amyloidosis, kezel\u00e9s\u00e9ben a kemoter\u00e1pi\u00e1nak \u00e9s \u0151ssejt-transzplant\u00e1ci\u00f3nak jut szerep. A m\u00e1sik gyakori t\u00edpus a kr\u00f3nikus fert\u0151z\u0151 vagy gyullad\u00e1sos betegs\u00e9gekhez, ritka \u00f6r\u00f6kletes l\u00e1z szindr\u00f3m\u00e1khoz t\u00e1rsul\u00f3 amyloidosis. A fibrillumok a sz\u00e9rum-amyloid-A-b\u00f3l k\u00e9pz\u0151dnek, amelynek szintje a C-reakt\u00edv proteinnel p\u00e1rhuzamosan emelkedik. A kezel\u00e9s f\u0151 pill\u00e9re az alapbetegs\u00e9g befoly\u00e1sol\u00e1sa, illetve a fibrillumok degrad\u00e1ci\u00f3j\u00e1nak el\u0151seg\u00edt\u00e9se. Ritk\u00e1bbak az \u00f6r\u00f6kletes amyloidosisform\u00e1k, ezekben a mut\u00e1ci\u00f3t szenvedett prekurzort\u00f3l f\u00fcgg\u0151en m\u00e1j-, m\u00e1j- \u00e9s vese-, valamint m\u00e1j- \u00e9s sz\u00edvtranszplant\u00e1ci\u00f3 j\u00f6n sz\u00f3ba.<\/jats:p>","DOI":"10.1556\/oh.2010.28919","type":"journal-article","created":{"date-parts":[[2010,7,1]],"date-time":"2010-07-01T05:06:52Z","timestamp":1277960812000},"page":"1182-1189","source":"Crossref","is-referenced-by-count":5,"title":["Amyloidosis"],"prefix":"10.1556","volume":"151","author":[{"given":"Csilla","family":"Trinn","sequence":"first","affiliation":[{"name":"1 Debreceni Egyetem, Orvos- \u00e9s Eg\u00e9szs\u00e9gtudom\u00e1nyi Centrum Belgy\u00f3gy\u00e1szati Int\u00e9zet, I. 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