{"id":53,"date":"2023-11-06T10:45:33","date_gmt":"2023-11-06T10:45:33","guid":{"rendered":"https:\/\/liveunlimitedpku-dev-001.azurewebsites.net\/hu-hu\/?page_id=53"},"modified":"2024-12-07T20:24:32","modified_gmt":"2024-12-07T20:24:32","slug":"minek-kell-megvaltoznia","status":"publish","type":"page","link":"https:\/\/pku.biomarin.com\/hu-hu\/liveunlimited\/minek-kell-megvaltoznia\/","title":{"rendered":"Minek kell megvaltoznia?"},"content":{"rendered":"
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\n\t\t\t \t\t\t\t Az \u00e9let bonyolult, ha nincsenek meg a sz\u00fcks\u00e9ges eszk\u00f6z\u00f6k<\/span>\n\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t

MINEK KELL MEGV\u00c1LTOZNIA?\n<\/h1>\n\t\t\t\t\t\t\t\t\t\t\t\t

Felsz\u00f3lalunk annak \u00e9rdek\u00e9ben, hogy megfelel\u0151 sz\u00e1m\u00fa feln\u0151tt anyagcsere specialista, dietetikus \u00e9s pszichol\u00f3gus \u00e1lljon rendelkez\u00e9sre minden PKU-val diagnosztiz\u00e1lt feln\u0151tt sz\u00e1m\u00e1ra Eur\u00f3p\u00e1ban.<\/p>\n\t\t\t\t\t\t\t\t\n

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A PKU<\/a> jelent\u0151s hat\u00e1ssal lehet egy ember \u00e9letmin\u0151s\u00e9g\u00e9re, de mivel az \u00e1llapot ritka \u00e9s nem \u00e1ltal\u00e1nosan ismert, az eur\u00f3pai eg\u00e9szs\u00e9g\u00fcgyi hat\u00f3s\u00e1gok jelenleg nem priorit\u00e1sk\u00e9nt tartj\u00e1k nyilv\u00e1n1<\/sup>.<\/h2>\n

M\u00e1ra \u00faj korszakba l\u00e9pt\u00fcnk a PKU tekintet\u00e9ben, ahol az els\u0151 feln\u0151ttek, akiknek sz\u00fclet\u00e9s\u00fckkor v\u00e9gzett tesztje az pozit\u00edv volt, eg\u00e9sz \u00e9let\u00fckben megkapj\u00e1k a sz\u00fcks\u00e9ges gondoskod\u00e1st. Ez \u00faj kih\u00edv\u00e1sokat jelent; jelenleg s\u00fcrg\u0151sen \u00faj megold\u00e1sokat kell tal\u00e1lnunk a gondoskod\u00e1s kialak\u00edt\u00e1s\u00e1ra \u00e9s a t\u00e1mogat\u00e1s biztos\u00edt\u00e1s\u00e1ra minden PKU-val \u00e9l\u0151 sz\u00e1m\u00e1ra.<\/p>\n

Az Eur\u00f3pai Fenilketonuria \u00e9s Fenilketonuriak\u00e9nt Kezelt Kapcsolt Rendelleness\u00e9gek T\u00e1rsas\u00e1ga (ESPKU) \u00e1ltal 2015-ben v\u00e9gzett kutat\u00e1sok \u00e9s a legfrissebb eur\u00f3pai klinikai ir\u00e1nyelvek (2017) alapj\u00e1n az \u00c9lj Teljes \u00c9letet kamp\u00e1ny felsz\u00f3lal a feln\u0151tt PKU betegek gondoz\u00e1s\u00e1hoz tartoz\u00f3 egyes konkr\u00e9t ter\u00fcletek olyan m\u00e9rv\u0171 jav\u00edt\u00e1sa \u00e9rdek\u00e9ben, amely megfelel\u0151en t\u00fckr\u00f6zi az \u00e1llapot \u00e9lethosszig tart\u00f3 term\u00e9szet\u00e9t.<\/p>\n <\/div>\n <\/div>\n <\/div>\n<\/div>\n\n

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Egys\u00e9gesebb kezel\u00e9st a PKU betegek kezel\u00e9s\u00e9ben Eur\u00f3pa-szerte\n<\/h3>\n

Az eur\u00f3pai ir\u00e1nyelvek vil\u00e1gos norm\u00e1kat fektetnek le a PKU kontroll\u00e1l\u00e1s\u00e1ra. Ahol nincsenek nemzeti ir\u00e1nyelvek, ott ezeket kell marad\u00e9ktalanul bevezetni annak \u00e9rdek\u00e9ben, hogy a PKU-val \u00e9l\u0151k hozz\u00e1jussanak a nekik sz\u00fcks\u00e9ges t\u00e1mogat\u00e1shoz \u00e9s gondoskod\u00e1shoz2<\/sup>.<\/p>\n <\/div>\n<\/div>\n\n

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Rendszeres hozz\u00e1f\u00e9r\u00e9s multidiszciplin\u00e1ris eg\u00e9szs\u00e9g\u00fcgyi szak\u00e9rt\u0151i csoporthoz\n<\/h3>\n

A PKU neurol\u00f3giai hat\u00e1sai \u00e9s a Phe szinten tart\u00e1sa \u00e9rdek\u00e9ben v\u00e1llalt rendk\u00edv\u00fcl szigor\u00fa \u00e9trend dac\u00e1ra \u2013 tanulm\u00e1nyok tan\u00fas\u00e1ga szerint \u2013 az\u00a0eur\u00f3pai \u00e9rintetteknek csup\u00e1n 12%-a f\u00e9r hozz\u00e1 egy\u00a0<\/strong>szakorvosokb\u00f3l, t\u00e1pl\u00e1lkoz\u00e1si szak\u00e9rt\u0151kb\u0151l, szak\u00e1pol\u00f3kb\u00f3l, pszichol\u00f3gusokb\u00f3l \u00e9s klinikai biok\u00e9mikusokb\u00f3l \u00e1ll\u00f3\u00a0multidiszciplin\u00e1ris csoport\u00a0<\/strong>seg\u00edts\u00e9g\u00e9hez3<\/sup>.<\/p>\n <\/div>\n<\/div>\n\n

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Fokozott t\u00e1mogat\u00e1s a gyermekkori gondoz\u00e1sb\u00f3l a feln\u0151ttkori gondoz\u00e1sba \u00e1tl\u00e9p\u0151k sz\u00e1m\u00e1ra\n<\/h3>\n

M\u00edg a PKU-val \u00e9l\u0151 gyermekek \u00e1ltal\u00e1ban havonta kapnak szakorvosi seg\u00edts\u00e9get, a feln\u0151ttek jellemz\u0151en csak \u00e9vente egyszer vagy m\u00e9g ritk\u00e1bban kapj\u00e1k meg ezt a lehet\u0151s\u00e9get2,3<\/sup>.<\/p>\n

18 \u00e9ves kor ut\u00e1n sok PKU-val \u00e9l\u0151 teljesen felhagy a szak\u00e9rt\u0151i konzult\u00e1ci\u00f3val, gyakran az\u00e9rt, mert \u00fagy l\u00e1tja, hogy nem kap klinikai seg\u00edts\u00e9get, illetve az \u00e9trendj\u00e9vel kapcsolatos f\u00e1sults\u00e1g miatt4<\/sup>.<\/p>\n

Azt hissz\u00fck, hogy a PKU jobban kontroll\u00e1lhat\u00f3, ha feln\u0151ttkorban is rendszeres szakorvosi figyelemben r\u00e9szes\u00fcl, \u00e9s a gyermekkori gondoz\u00e1sb\u00f3l a feln\u0151ttkori gondoz\u00e1sba val\u00f3 \u00e1tl\u00e9p\u00e9skor egy\u00e9nre szabott gondoz\u00e1si terv k\u00e9sz\u00fcl.<\/p>\n <\/div>\n<\/div>\n\t\t\t<\/div>\n\t\t<\/div>\n\t<\/div>\n<\/div>\n\n

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Referenci\u00e1k:\n<\/h4>\n\t\t\t\t\t\t
    \n
  1. PKU: Closing the Gaps in Care an ESPKU benchmark report on the management of phenylketonuria within EU healthcare economies. (PKU: A h\u00e9zag megsz\u00fcntet\u00e9se; ESPKU referencia jelent\u00e9s a fenilketonuria kezel\u00e9s\u00e9r\u0151l az eur\u00f3pai eg\u00e9szs\u00e9g\u00fcgyi rendszerekben)\n<\/span><\/li>\n
  2. Wegberg AMJ, MacDonald A, Ahring K, \u00e9s m\u00e1sok. The complete European guidelines on phenylketonuria: diagnosis and treatment. (A fenilketonuri\u00e1val kapcsolatos \u00f6sszes Eur\u00f3pai Ir\u00e1nyelv) Orphanet J Rare Dis. 2017;12(1):162. 2017 okt\u00f3ber Orphanet J Rare Dis. 2017;12(1):162. Kiadva: 2017. okt. 12. doi:10.1186\/s13023-017-0685-2.\n<\/span><\/li>\n
  3. Blau \u00e9s m\u00e1sok. Management of phenylketonuria in Europe: Survey results from 19 countries. Molecular Genetics and Metabolism 99 (A fenilketonuria kezel\u00e9se Eur\u00f3p\u00e1ban: Felm\u00e9r\u00e9si eredm\u00e9nyek 19 orsz\u00e1gb\u00f3l. Molekul\u00e1ris genetika \u00e9s metabolizmus) (2010) 109\u2013115\n<\/span><\/li>\n
  4. Thomas J \u00e9s m\u00e1sok. Strategies for Successful Long-Term Engagement of Adults With Phenylalanine Hydroxylase Deficiency Returning to the Clinic Journal of Inborn Errors of Metabolism & Screening 2017, Volume 5: 1\u20139, 2017 (A klinik\u00e1ra visszat\u00e9r\u0151 fenilalanin hidroxil\u00e1z hi\u00e1nyos feln\u0151ttek sikeres bevon\u00e1si strat\u00e9gi\u00e1ja hossz\u00fat\u00e1v\u00fa ell\u00e1t\u00e1sba; Velesz\u00fcletett anyagcsere hib\u00e1k \u00e9s sz\u0171r\u00e9s\u00fck \u2013 Szaklap, 2017, 5. k\u00f6tet: 1-9, 2017.)\n<\/span><\/li>\n
  5. Cazzorla C \u00e9s m\u00e1sok. Living with phenylketonuria in adulthood: The PKU ATTITUDE study. Molecular Genetics and Metabolism Reports 16 (Fenilketonuri\u00e1val \u00e9lni feln\u0151ttkorban: A PKU magatart\u00e1s. Molekul\u00e1ris genetika \u00e9s metabolizmus jelent\u00e9sek) (2018) 39\u201345\n<\/span><\/li>\n <\/ol>\n\t\t\t\t\t<\/div>\n\t<\/div>\n<\/div>","protected":false},"excerpt":{"rendered":"","protected":false},"author":2,"featured_media":0,"parent":0,"menu_order":0,"comment_status":"closed","ping_status":"closed","template":"","meta":{"_acf_changed":false,"inline_featured_image":false,"footnotes":""},"class_list":["post-53","page","type-page","status-publish","hentry"],"acf":[],"yoast_head":"\nMinek kell megv\u00e1ltoznia? | Live Unlimited PKU<\/title>\n<meta name=\"description\" content=\"S\u00fcrg\u0151sen meg kell tal\u00e1lnunk a m\u00f3dj\u00e1t az ell\u00e1t\u00e1s fejleszt\u00e9s\u00e9nek \u00e9s a PKU-val \u00e9l\u0151k sz\u00fcks\u00e9gleteinek t\u00e1mogat\u00e1s\u00e1nak.\" \/>\n<meta name=\"robots\" content=\"index, follow, max-snippet:-1, max-image-preview:large, max-video-preview:-1\" \/>\n<link rel=\"canonical\" href=\"https:\/\/pku.biomarin.com\/hu-hu\/liveunlimited\/minek-kell-megvaltoznia\/\" \/>\n<meta property=\"og:locale\" content=\"hu_HU\" \/>\n<meta property=\"og:type\" content=\"article\" \/>\n<meta property=\"og:title\" content=\"Minek kell megv\u00e1ltoznia? 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