{"id":1830,"date":"2023-02-20T16:00:00","date_gmt":"2023-02-20T15:00:00","guid":{"rendered":"https:\/\/niho.sk\/?p=1830"},"modified":"2024-03-30T16:09:09","modified_gmt":"2024-03-30T15:09:09","slug":"22b-liecivo-emicizumab-hemlibra-pri-profylaxii-krvacania-u-pacientov-vo-vsetkych-vekovych-skupinach-s-tazkou-hemofiliou-a-bez-inhibitora-faktora-viii","status":"publish","type":"post","link":"https:\/\/www.niho.sk\/en\/22b-liecivo-emicizumab-hemlibra-pri-profylaxii-krvacania-u-pacientov-vo-vsetkych-vekovych-skupinach-s-tazkou-hemofiliou-a-bez-inhibitora-faktora-viii\/","title":{"rendered":"22B: Lie\u010divo emicizumab (Hemlibra) pri profylaxii krv\u00e1cania u pacientov vo v\u0161etk\u00fdch vekov\u00fdch skupin\u00e1ch s \u0165a\u017ekou hemof\u00edliou A bez inhib\u00edtora faktora VIII"},"content":{"rendered":"
    \n
  1. Ak\u00e1 je z\u00e1\u0165a\u017e ochorenia pre pacienta? Ako z\u00e1sadn\u00e9 je ochorenie?<\/strong><\/li>\n<\/ol>\n\n\n\n

    Hemof\u00edlia typu A je pomerne vz\u00e1cne vroden\u00e9 nevylie\u010dite\u013en\u00e9 ochorenie, postihuj\u00face predov\u0161etk\u00fdm mu\u017eov. Prejavuje sa poruchou zr\u00e1\u017eania krvi, sp\u00f4sobenou nedostatkom faktora zr\u00e1\u017eania VIII. Pacienti s hemof\u00edliou krv\u00e1caj\u00fa po poranen\u00ed alebo pri oper\u00e1cii dlh\u0161ie ako zdrav\u00ed \u013eudia. Lie\u010dba v s\u00fa\u010dasnosti spo\u010d\u00edva v nahraden\u00ed ch\u00fdbaj\u00facich faktorov zr\u00e1\u017eania. Pribli\u017ene u 30% pacientov sa vyvin\u00fa inhib\u00edtory proti faktoru VIII (FVIII), ktor\u00e9 neutralizuj\u00fa pod\u00e1van\u00fd FVIII a robia lie\u010dbu ne\u00fa\u010dinnou.<\/p>\n\n\n\n

    U \u0165a\u017ek\u00fdch hemofilikov \u010dastokr\u00e1t doch\u00e1dza ku krv\u00e1caniu, ktor\u00e9 sa ozna\u010duje ako spont\u00e1nne, bez zjavnej pr\u00ed\u010diny. Spont\u00e1nne krv\u00e1canie sa nikdy ned\u00e1 predv\u00edda\u0165 a ned\u00e1 sa mu \u00faplne pred\u00eds\u0165. V\u0161etci pacienti s potvrdenou \u0165a\u017ekou hemof\u00edliou A si vy\u017eaduj\u00fa lie\u010dbu. Bez lie\u010dby vedie krv\u00e1canie do k\u013abov a svalov ku vzniku degenerat\u00edvnych zmien v k\u013aboch, k tzv. progres\u00edvnej artropatii a k atrofii svalstva (najm\u00e4 kon\u010datinov\u00e9ho). Aj jedin\u00e9 krv\u00e1canie m\u00f4\u017ee vies\u0165 k \u017eivot ohrozuj\u00facemu stavu alebo trval\u00e9mu oslabuj\u00facemu ochoreniu k\u013abov.<\/p>\n\n\n\n

      \n
    1. O ak\u00fd liek ide?<\/strong><\/li>\n<\/ol>\n\n\n\n

      Hemlibra obsahuje lie\u010divo \u201eemicizumab\u201c, ktor\u00fd patr\u00ed do skupiny liekov naz\u00fdvan\u00fdch \u201emonoklon\u00e1lne protil\u00e1tky\u201c. Monoklon\u00e1lne protil\u00e1tky s\u00fa typom bielkov\u00edn, ktor\u00e9 rozpoznaj\u00fa \u0161pecifick\u00fd cie\u013e v tele a navia\u017eu sa na\u0148. <\/p>\n\n\n\n

      \u00dalohou Hemlibry je premos\u0165ova\u0165 priestor medzi aktivovan\u00fdm faktorom IX a faktorom X, aby do\u0161lo k obnoveniu funkcie ch\u00fdbaj\u00faceho aktivovan\u00e9ho faktora VIII, ktor\u00fd je potrebn\u00fd na zastavenie krv\u00e1cania. Hemlibra m\u00e1 \u0161trukt\u00faru odli\u0161n\u00fa od \u0161trukt\u00fary faktora VIII, a preto pr\u00edtomnos\u0165 inhib\u00edtora faktora VIII nem\u00e1 vplyv na jej p\u00f4sobenie.<\/p>\n\n\n\n

      Hemlibra sa m\u00e1 pou\u017e\u00edva\u0165 na predch\u00e1dzanie vzniku krv\u00e1can\u00ed (profylaktick\u00e1 lie\u010dba).<\/p>\n\n\n\n

      Liek Hemlibra bol registrovan\u00fd Eur\u00f3pskou liekovou agent\u00farou vo febru\u00e1ri 2018.<\/p>\n\n\n\n

        \n
      1. Ako sa na emicizumab pozeraj\u00fa odborn\u00edci? <\/strong><\/li>\n<\/ol>\n\n\n\n

        Odborn\u00edk, ktor\u00e9ho NIHO oslovilo, pova\u017euje za relevantn\u00e9 klinick\u00e9 ukazovatele po\u010det spont\u00e1nnych krv\u00e1can\u00ed a stav k\u013abov\u00e9ho apar\u00e1tu. Za klinicky v\u00fdznamn\u00fa odpove\u010f na lie\u010dbu by pova\u017eoval nulov\u00e9 ro\u010dn\u00e9 spont\u00e1nne krv\u00e1canie. Podobne ako druh\u00fd odborn\u00edk vid\u00ed pr\u00ednos lieku Hemlibra pre pacientov s hemof\u00edliou A s inhib\u00edtorom aj bez inhib\u00edtora, pre pacientov, ktor\u00ed maj\u00fa napriek \u0161tandardnej lie\u010dbe \u010dast\u00e9 krv\u00e1cania a pacientov s nedostato\u010dn\u00fdm \u017eilov\u00fdm pr\u00edstupom.<\/p>\n\n\n\n

        Odborn\u00edci vidia pr\u00ednos v zn\u00ed\u017een\u00ed krv\u00e1can\u00ed aj sp\u00f4sobe aplik\u00e1cie lieku Hemlibra.<\/p>\n\n\n\n

          \n
        1. \u010co hodnotilo NIHO a ak\u00fd je v\u00fdsledok?<\/strong><\/li>\n<\/ol>\n\n\n\n

          Dr\u017eite\u013e registr\u00e1cie (Roche) po\u017eaduje hradenie lieku z prostriedkov verejn\u00e9ho zdravotn\u00e9ho poistenia pri prevenci\u00ed krv\u00e1cania u pacientov vo v\u0161etk\u00fdch vekov\u00fdch skupin\u00e1ch s \u0165a\u017ekou hemof\u00edliou A bez inhib\u00edtora faktora VIII.<\/p>\n\n\n\n

          NIHO odpor\u00fa\u010da nevyhovie\u0165 <\/strong>\u017eiadosti o kategorizovanie lieku Hemlibra v po\u017eadovanej indik\u00e1cii. Dr\u017eite\u013e registr\u00e1cie nepreuk\u00e1zal n\u00e1kladov\u00fa efekt\u00edvnos\u0165 lie\u010diva emicizumab vo\u010di v\u0161etk\u00fdm relevantn\u00fdm kompar\u00e1torom pod\u013ea \u00a77 ods. 1 p\u00edsm. f) z\u00e1kona \u010d. 363\/2011 Z.z.. Nako\u013eko sa dr\u017eite\u013e registr\u00e1cie odmietol porovna\u0165 vo\u010di v\u0161etk\u00fdm relevantn\u00fdm kompar\u00e1torom, nebolo mo\u017en\u00e9 zhodnoti\u0165 n\u00e1kladov\u00fa efekt\u00edvnos\u0165 prevencie emicizumabu.<\/p>","protected":false},"excerpt":{"rendered":"

          NIHO odpor\u00fa\u010da nevyhovie\u0165 \u017eiadosti o kategorizovanie lieku Hemlibra v po\u017eadovanej indik\u00e1cii. Dr\u017eite\u013e registr\u00e1cie nepreuk\u00e1zal n\u00e1kladov\u00fa efekt\u00edvnos\u0165 …<\/p>","protected":false},"author":3,"featured_media":1828,"comment_status":"closed","ping_status":"closed","sticky":false,"template":"","format":"standard","meta":{"inline_featured_image":false,"footnotes":""},"categories":[11],"tags":[80,79],"class_list":["post-1830","post","type-post","status-publish","format-standard","has-post-thumbnail","hentry","category-sumar-hodnotenia","tag-emicizumab","tag-hemlibra"],"_links":{"self":[{"href":"https:\/\/www.niho.sk\/en\/wp-json\/wp\/v2\/posts\/1830","targetHints":{"allow":["GET"]}}],"collection":[{"href":"https:\/\/www.niho.sk\/en\/wp-json\/wp\/v2\/posts"}],"about":[{"href":"https:\/\/www.niho.sk\/en\/wp-json\/wp\/v2\/types\/post"}],"author":[{"embeddable":true,"href":"https:\/\/www.niho.sk\/en\/wp-json\/wp\/v2\/users\/3"}],"replies":[{"embeddable":true,"href":"https:\/\/www.niho.sk\/en\/wp-json\/wp\/v2\/comments?post=1830"}],"version-history":[{"count":0,"href":"https:\/\/www.niho.sk\/en\/wp-json\/wp\/v2\/posts\/1830\/revisions"}],"wp:featuredmedia":[{"embeddable":true,"href":"https:\/\/www.niho.sk\/en\/wp-json\/wp\/v2\/media\/1828"}],"wp:attachment":[{"href":"https:\/\/www.niho.sk\/en\/wp-json\/wp\/v2\/media?parent=1830"}],"wp:term":[{"taxonomy":"category","embeddable":true,"href":"https:\/\/www.niho.sk\/en\/wp-json\/wp\/v2\/categories?post=1830"},{"taxonomy":"post_tag","embeddable":true,"href":"https:\/\/www.niho.sk\/en\/wp-json\/wp\/v2\/tags?post=1830"}],"curies":[{"name":"wp","href":"https:\/\/api.w.org\/{rel}","templated":true}]}}