{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2024,1,29]],"date-time":"2024-01-29T10:57:01Z","timestamp":1706525821058},"reference-count":19,"publisher":"Akademiai Kiado Zrt.","issue":"21","license":[{"start":{"date-parts":[[2022,5,22]],"date-time":"2022-05-22T00:00:00Z","timestamp":1653177600000},"content-version":"unspecified","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["akjournals.com"],"crossmark-restriction":true},"short-container-title":["OH"],"published-print":{"date-parts":[[2022,5,22]]},"abstract":"<jats:p>A cystinosis ritka, aminosav-anyagcser\u00e9t \u00e9rint\u0151, lizoszom\u00e1lis t\u00e1rol\u00e1si\n                    megbeteged\u00e9s, melyben jellegzetes elt\u00e9r\u00e9s a cisztin felszaporod\u00e1sa \u00e9s\n                    kikrist\u00e1lyosod\u00e1sa a k\u00fcl\u00f6nb\u00f6z\u0151 szervekben, sz\u00f6vetekben, els\u0151sorban a szemben \u00e9s a\n                    ves\u00e9ben. Szem\u00e9szeti vonatkoz\u00e1sban kiemelked\u0151 jelent\u0151s\u00e9g\u0171 a cisztinnek a\n                    corne\u00e1ban val\u00f3 lerak\u00f3d\u00e1sa, ami nagyfok\u00fa f\u00e9ny\u00e9rz\u00e9kenys\u00e9g kialakul\u00e1s\u00e1hoz vezet. A\n                    kifejezett f\u00e9ny\u00e9rz\u00e9kenys\u00e9g jelent\u0151sen rontja az \u00e9rintett p\u00e1ciensek\n                    \u00e9letmin\u0151s\u00e9g\u00e9t, \u00edgy a krist\u00e1lyok mennyis\u00e9g\u00e9nek cs\u00f6kkent\u00e9s\u00e9t c\u00e9lz\u00f3 hat\u00e9kony\n                    szem\u00e9szeti kezel\u00e9s kiemelt szereppel b\u00edr. Esettanulm\u00e1nyunk kapcs\u00e1n a\n                    szakirodalom \u00e1ttekint\u00e9s\u00e9vel mutatjuk be a cystinosis jellegzetes szem\u00e9szeti\n                    klinikai megjelen\u00e9s\u00e9t \u00e9s kezel\u00e9si lehet\u0151s\u00e9geit. A lok\u00e1lis kezel\u00e9sben kor\u00e1bban\n                    sz\u00e9les k\u00f6rben alkalmazt\u00e1k a krist\u00e1lyok felold\u00e1s\u00e1t el\u0151seg\u00edt\u0151 ciszteamin egyszer\u0171\n                    vizes oldat\u00e1t, e k\u00e9sz\u00edtm\u00e9ny ter\u00e1pi\u00e1s hat\u00e9konys\u00e1ga azonban vitatott. \u00dajabban a\n                    ciszteamin \u00faj, viszk\u00f3zus oldat\u00e1nak alkalmaz\u00e1s\u00e1ra ny\u00edlt lehet\u0151s\u00e9g. A 29 \u00e9ves\n                    f\u00e9rfi beteg\u00fcnkn\u00e9l a cornealis cisztinkrist\u00e1lyok kezel\u00e9s\u00e9re viszk\u00f3zus form\u00e1tum\u00fa,\n                    ciszteamintartalm\u00fa k\u00e9sz\u00edtm\u00e9nyt vezett\u00fcnk be, \u00e9s ezt alkalmaztuk 1 \u00e9ven \u00e1t.\n                    P\u00e1ciens\u00fcnkn\u00e9l az \u00faj lok\u00e1lis szemcsepp haszn\u00e1lata mellett a kezel\u00e9s megkezd\u00e9s\u00e9t\n                    k\u00f6vet\u0151 egy \u00e9vben a f\u00e9ny\u00e9rz\u00e9kenys\u00e9g jelent\u0151s, fokozatos cs\u00f6kken\u00e9s\u00e9t figyelt\u00fck\n                    meg. El\u00fcls\u0151 szegmens optikaikoherencia-tomogr\u00e1fi\u00e1s \u00e9s <jats:italic>in vivo<\/jats:italic>\n                    konfok\u00e1lis mikroszk\u00f3pos vizsg\u00e1latainkkal kimutathat\u00f3 volt a cornealis krist\u00e1lyok\n                    mennyis\u00e9g\u00e9nek folyamatos cs\u00f6kken\u00e9se m\u00e1r az 1. h\u00f3napt\u00f3l kezdve. Jelen\n                    k\u00f6zlem\u00e9ny\u00fcnk c\u00e9lja, hogy felh\u00edvja a figyelmet a cystinosis szem\u00e9szeti t\u00fcneteire,\n                    illetve a Magyarorsz\u00e1gon els\u0151k\u00e9nt alkalmazott, viszk\u00f3zus form\u00e1tum\u00fa\n                    ciszteaminkezel\u00e9s hat\u00e9konys\u00e1g\u00e1ra. Orv Hetil. 2022; 163(21): 846\u2013852.<\/jats:p>","DOI":"10.1556\/650.2022.32484","type":"journal-article","created":{"date-parts":[[2022,11,18]],"date-time":"2022-11-18T13:36:23Z","timestamp":1668778583000},"page":"846-852","update-policy":"http:\/\/dx.doi.org\/10.1556\/policypage.crossmark.crossref.1","source":"Crossref","is-referenced-by-count":1,"title":["Ciszteamintartalm\u00fa szemcsepp alkalmaz\u00e1sa cystinosisban"],"prefix":"10.1556","volume":"163","author":[{"given":"Anita","family":"Csorba","sequence":"first","affiliation":[{"name":"Semmelweis Egyetem, \u00c1ltal\u00e1nos Orvostudom\u00e1nyi Kar, Szem\u00e9szeti Klinika Budapest, M\u00e1ria u. 39.; 1085 Magyarorsz\u00e1g"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Erika","family":"Maka","sequence":"additional","affiliation":[{"name":"Semmelweis Egyetem, \u00c1ltal\u00e1nos Orvostudom\u00e1nyi Kar, Szem\u00e9szeti Klinika Budapest, M\u00e1ria u. 39.; 1085 Magyarorsz\u00e1g"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Attila","family":"Szab\u00f3","sequence":"additional","affiliation":[{"name":"Semmelweis Egyetem, \u00c1ltal\u00e1nos Orvostudom\u00e1nyi Kar, I. 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