{"id":2171,"date":"2022-03-31T17:28:40","date_gmt":"2022-03-31T16:28:40","guid":{"rendered":"https:\/\/slanedeti.sk\/cms\/?p=2171"},"modified":"2022-03-31T17:28:41","modified_gmt":"2022-03-31T16:28:41","slug":"tehotenstvo-a-cysticka-fibroza","status":"publish","type":"post","link":"https:\/\/slanedeti.sk\/cms\/tehotenstvo-a-cysticka-fibroza\/2171","title":{"rendered":"Tehotenstvo a cystick\u00e1 fibr\u00f3za"},"content":{"rendered":"\n<p>Lie\u010dba cystickej fibr\u00f3zy (CF) sa za posledn\u00fdch 50 rokov dramaticky zmenila v\u010faka skr\u00edningu, skor\u00e9mu zavedeniu \u00fa\u010dinnej lie\u010dby respira\u010dn\u00fdch infekci\u00ed a pr\u00e1ci \u0161pecializovan\u00fdch centier. Skr\u00edning umo\u017e\u0148uje v\u010dasn\u00fa intervenciu v pr\u00edpade \u010fal\u0161\u00edch komplik\u00e1ci\u00ed vr\u00e1tane cukrovky, ochorenia pe\u010dene a osteopor\u00f3zy. Dnes je be\u017en\u00e9, \u017ee sa pacienti do\u017e\u00edvaj\u00fa dospelosti. V\u0161etky tieto aspekty prispievaj\u00fa k\u00a0tomu, \u017ee sa aj \u00a0CF pacienti m\u00f4\u017eu sta\u0165 rodi\u010dmi. Vo svete s\u00fa zn\u00e1me aj viacpo\u010detn\u00e9 tehotenstv\u00e1. N\u00e1ro\u010dnos\u0165 tehotenstva u\u00a0jednotliv\u00fdch \u017eien z\u00e1vis\u00ed od toho, ko\u013eko negat\u00edvnych\u00a0 faktorov ovplyv\u0148uje ich tehotenstvo:<\/p>\n\n\n\n<ul class=\"wp-block-list\"><li>P\u013e\u00facne funkcie<\/li><li>BMI Index<\/li><li>Diabetes<\/li><li>Lie\u010dba modul\u00e1tormi CFTR<\/li><li>Transplantovan\u00e9 p\u013e\u00faca<\/li><li>\u010eal\u0161ie faktory<\/li><\/ul>\n\n\n\n<figure class=\"wp-block-image size-large\"><a href=\"https:\/\/slanedeti.sk\/cms\/wp-content\/uploads\/2022\/03\/tehotenstvo_2.jpg\"><img loading=\"lazy\" decoding=\"async\" width=\"1024\" height=\"682\" src=\"https:\/\/slanedeti.sk\/cms\/wp-content\/uploads\/2022\/03\/tehotenstvo_2-1024x682.jpg\" alt=\"tehotenstvo\" class=\"wp-image-2172\" srcset=\"https:\/\/slanedeti.sk\/cms\/wp-content\/uploads\/2022\/03\/tehotenstvo_2-1024x682.jpg 1024w, https:\/\/slanedeti.sk\/cms\/wp-content\/uploads\/2022\/03\/tehotenstvo_2-300x200.jpg 300w, https:\/\/slanedeti.sk\/cms\/wp-content\/uploads\/2022\/03\/tehotenstvo_2-768x512.jpg 768w, https:\/\/slanedeti.sk\/cms\/wp-content\/uploads\/2022\/03\/tehotenstvo_2.jpg 1280w\" sizes=\"auto, (max-width: 1024px) 100vw, 1024px\" \/><\/a><\/figure>\n\n\n\n<h4 class=\"wp-block-heading\">P\u013e\u00facne funkcie a\u00a0BMI Index<\/h4>\n\n\n\n<p>Prv\u00fdm faktorom, ktor\u00fd z\u00e1sadne ovplyv\u0148uje mo\u017enos\u0165 ma\u0165 die\u0165a, s\u00fa p\u013e\u00facne funkcie a\u00a0BMI index.\u00a0 Predpoklad\u00e1 sa, \u017ee zl\u00e1 funkcia p\u013e\u00fac v \u010dase po\u010datia je najpredikt\u00edvnej\u0161\u00edm faktorom ur\u010duj\u00facim v\u00fdsledok tehotenstva. Odborn\u00edci sa vo viacer\u00fdch \u0161t\u00fadi\u00e1ch zhoduj\u00fa na rozdelen\u00ed bud\u00facich matiek do skup\u00edn:<\/p>\n\n\n\n<ul class=\"wp-block-list\"><li>s&nbsp;miernym ochoren\u00edm (ppFEV 1 &gt; 60 % a BMI &gt; 21)<\/li><li>so stredne \u0165a\u017ek\u00fdm ochoren\u00edm (ppFEV 1 &lt; 60 % \u00b1 BMI &lt; 21)<\/li><\/ul>\n\n\n\n<p>Matky s miernym ochoren\u00edm mali ur\u010dit\u00fa obnovu FEV 1 &#8222;straten\u00e9ho&#8220; po\u010das tehotenstva, zatia\u013e \u010do stredne \u0165a\u017ek\u00e1 skupina po tehotenstve \u010falej str\u00e1cala p\u013e\u00facne funkcie (p &lt; 0,05). [1]\n\n\n\n<h4 class=\"wp-block-heading\">Diabetes<\/h4>\n\n\n\n<p>\u010eal\u0161\u00edm v\u00fdrazn\u00fdm faktorom ovplyv\u0148uj\u00facim tehotenstvo je diabetes (cukrovka). Diabetes u tehotn\u00fdch \u017eien s CF je naj\u010dastej\u0161ie bu\u010f gesta\u010dn\u00fd diabetes mellitus (GDM) alebo pregesta\u010dn\u00fd diabetes s\u00favisiaci s cystickou fibr\u00f3zou (CFRD). GDM je pod\u013ea Americkej diabetologickej spolo\u010dnosti (ADA) z\u00a0roku 2017\u00a0definovan\u00fd ako diabetes zachyten\u00fd v\u00a02. alebo 3. trimestri gravidity u\u00a0\u017eien bez anamn\u00e9zy diabetes mellitus pred graviditou. T\u00e1to porucha typicky odznieva v\u00a0priebehu \u0161estonedelia. \u00a0Prevalencia diabetu v tehotenstve je vysok\u00e1 a v priebehu \u010dasu st\u00fapa. Toto zvy\u0161ovanie prevalencie m\u00e1 viacero pr\u00ed\u010din. Prv\u00fdm faktorom je zvy\u0161uj\u00faca sa d\u013a\u017eka \u017eivota \u2013 \u010doraz viac \u017eien s CF sa do\u017eije dospel\u00e9ho veku. A\u017e 40-50% dospel\u00fdch s CF m\u00e1 CFRD a u \u017eien vo veku 30-39 rokov je zv\u00fd\u0161en\u00e1 prevalencia v porovnan\u00ed s mlad\u0161\u00edmi a star\u0161\u00edmi vekov\u00fdmi skupinami zdrav\u00fdch \u017eien. U v\u00fdznamnej \u010dasti \u017eien s CF sa CFRD vyvinie po\u010das ich reproduk\u010dn\u00e9ho obdobia. Druh\u00fdm faktorom je, \u017ee aj ke\u010f \u017eena s CF nem\u00e1 pred graviditou CFRD, GDM je u \u017eien s CF \u010dastej\u0161\u00ed. Tehotenstvo je fyziologicky ve\u013emi n\u00e1ro\u010dn\u00e9 obdobie, v ktorom sa stup\u0148uje inzul\u00ednov\u00e1 rezistencia. Stup\u0148ovanie za\u010d\u00edna v druhom trimestri a vy\u017eaduje cca 50 % zv\u00fd\u0161enie produkcie inzul\u00ednu oproti hladin\u00e1m pred tehotenstvom. Ak sa toto zv\u00fd\u0161enie potreby inzul\u00ednu ned\u00e1 pokry\u0165, vznik\u00e1 GDM.\u00a0 U\u017e bolo dok\u00e1zan\u00e9, \u017ee tehotn\u00e9 \u017eeny s CF, ale bez preexistuj\u00facej CFRD, maj\u00fa ni\u017e\u0161iu produkciu inzul\u00ednu v porovnan\u00ed s tehotn\u00fdmi \u017eenami bez CF.\u00a0 Taktie\u017e bolo dok\u00e1zan\u00e9, \u017ee v porovnan\u00ed s netehotn\u00fdmi \u017eenami s\u00a0CF, maj\u00fa tehotn\u00e9 \u017eeny s CF zn\u00ed\u017een\u00fa citlivos\u0165 na inzul\u00edn a zv\u00fd\u0161en\u00fa produkciu gluk\u00f3zy v pe\u010deni, ale bez v\u00fdznamn\u00e9ho rozdielu v produkcii inzul\u00ednu . Poukazuje to na fakt, pre\u010do sa u \u017eien s CF vyskytuje tak\u00e1 vysok\u00e1 miera GDM v d\u00f4sledku kombin\u00e1cie zv\u00fd\u0161enej inzul\u00ednovej rezistencie a ni\u017e\u0161ej schopnosti zvy\u0161ova\u0165 produkciu inzul\u00ednu. [2]\n\n\n\n<h4 class=\"wp-block-heading\">Modul\u00e1tory CFTR<\/h4>\n\n\n\n<p>Prelomom v lie\u010dbe CF ju u\u017e\u00edvanie modul\u00e1torov CFTR. S\u00fa to n\u00edzkomolekulov\u00e9 lie\u010div\u00e1, ktor\u00e9 zlep\u0161uj\u00fa funkciu prote\u00ednu CFTR (cystic fibrosis transmembrane conductance regulator). S cie\u013eom poskytn\u00fa\u0165 \u010fal\u0161ie d\u00f4kazy o bezpe\u010dnosti modul\u00e1torov CFTR, sa uskuto\u010dnil medzin\u00e1rodn\u00fd prieskum medzi lek\u00e1rmi zaoberaj\u00facimi sa CF s cie\u013eom op\u00edsa\u0165 v\u00fdsledky u \u017eien s CF, ktor\u00e9 otehotneli po\u010das ich u\u017e\u00edvania.<\/p>\n\n\n\n<p>V rokoch 2018-2019 vedci vypracovali a zaslali dotazn\u00edk ved\u00facim lek\u00e1rom v centr\u00e1ch pre dospel\u00fdch pacientov s CF v USA, Spojenom kr\u00e1\u013eovstve, Izraeli, Eur\u00f3pe a Austr\u00e1lii, v ktorom \u017eiadali o anonymizovan\u00e9 \u00fadaje o v\u0161etk\u00fdch tehotenstv\u00e1ch \u017eien u\u017e\u00edvaj\u00facich modul\u00e1tory CFTR v \u010dase po\u010datia. Z\u00edskali&nbsp; \u00fadaje z 31 centier CF (17 USA, 5 Austr\u00e1lia, 4 Spojen\u00e9 kr\u00e1\u013eovstvo, 4 kontinent\u00e1lna Eur\u00f3pa, 1 Izrael), o 64 tehotenstv\u00e1ch u 61 \u017eien, ktor\u00fdch v\u00fdsledkom bolo 60 \u017eiv\u00fdch p\u00f4rodov. Medzi u\u017e\u00edvan\u00e9 modul\u00e1tory CFTR patrili Ivakaftor- IVA (31 tehotenstiev), Lumakaftor\/Ivacaftor- LUM\/IVA (26 tehotenstiev) a Tezakaftor\/Ivacaftor- TEZ\/IVA (7 tehotenstiev). D\u00f4kazy z tejto s\u00e9rie pr\u00edpadov nazna\u010duj\u00fa, \u017ee doteraz dostupn\u00e9 modul\u00e1tory CFTR (IVA, LUM\/IVA a TEZ\/IVA) s\u00fa v tehotenstve vo v\u0161eobecnosti dobre tolerovan\u00e9. V 64 zahrnut\u00fdch tehotenstv\u00e1ch boli hl\u00e1sen\u00e9 3 potraty v prvom trimestri, \u010do je miera, ktor\u00e1 je priazniv\u00e1 v porovnan\u00ed s o\u010dak\u00e1vanou mierou potratov v prvom trimestri u \u017eien v tomto vekovom rozmedz\u00ed (pribli\u017ene 10 %). Boli hl\u00e1sen\u00e9 dve matersk\u00e9 komplik\u00e1cie, ktor\u00e9 klinick\u00fd lek\u00e1r pova\u017eoval za s\u00favisiace s lie\u010dbou LUM\/IVA, hoci je diskutabiln\u00e9, \u010di tieto komplik\u00e1cie skuto\u010dne s\u00faviseli s modul\u00e1torom. V\u00e4\u010d\u0161ina komplik\u00e1ci\u00ed pozorovan\u00fdch po\u010das tehotenstva sa vyskytla u 9 \u017eien, ktor\u00e9 sa rozhodli ukon\u010di\u0165 lie\u010dbu IVA alebo LUM\/IVA po\u010das tehotenstva a n\u00e1sledne do\u0161lo k zhor\u0161eniu ich zdravotn\u00e9ho stavu v s\u00favislosti s CF. U t\u00fdch \u017eien, ktor\u00e9 sa rozhodli obnovi\u0165 lie\u010dbu nesk\u00f4r po\u010das tehotenstva, do\u0161lo k zlep\u0161eniu klinick\u00e9ho stavu a k \u017eiadnym zjavn\u00fdm komplik\u00e1ci\u00e1m. [3]\n\n\n\n<p>V&nbsp;E\u00da s\u00fa aktu\u00e1lne schv\u00e1len\u00e9&nbsp; nasledovn\u00e9 modul\u00e1tory CFTR:<\/p>\n\n\n\n<ul class=\"wp-block-list\"><li>Ivakaftor \u2013 Kalydeco<\/li><li>Kombin\u00e1cia Lumakaftoru a Ivakaftoru \u2013 Orkambi<\/li><li>Kombin\u00e1ciou tezakaftoru a ivakaftoru &#8211; Symkevi<\/li><li>Trojkombin\u00e1ca tezakaftoru, ivakaftoru a elexakaftoru \u2013 Kafrio<\/li><\/ul>\n\n\n\n<h4 class=\"wp-block-heading\">Tehotentstvo po transplant\u00e1cii p\u013e\u00fac<\/h4>\n\n\n\n<p>Najv\u00e1\u017enej\u0161ou prek\u00e1\u017ekou tehotenstva je transplant\u00e1cia p\u013e\u00fac. Aj po nej je v\u0161ak mo\u017en\u00e9 \u00faspe\u0161ne po\u010da\u0165, donosi\u0165 a porodi\u0165 die\u0165a. K tejto mo\u017enosti v\u0161ak treba pristupova\u0165 s mimoriadnou opatrnos\u0165ou, preto\u017ee tieto tehotenstv\u00e1 s\u00fa vysoko rizikov\u00e9. Sp\u00e1ja sa s nimi odmietnutie transplant\u00e1tu, nedonosenie die\u0165a\u0165a a n\u00edzka p\u00f4rodn\u00e1 hmotnos\u0165. Niektor\u00e9 transplanta\u010dn\u00e9 centr\u00e1 pre tieto rizik\u00e1 neodpor\u00fa\u010daj\u00fa \u017een\u00e1m po transplant\u00e1cii otehotnie\u0165. V t\u00fdchto pr\u00edpadoch je potrebn\u00e1 otvoren\u00e1 diskusia medzi odborn\u00fdmi t\u00edmami, ktor\u00e9 vyhodnotia rizik\u00e1 a pr\u00ednosy spojen\u00e9 s tehotenstvom. [4]\n\n\n\n<h4 class=\"wp-block-heading\">\u010eal\u0161ie faktory<\/h4>\n\n\n\n<p>Nemenej d\u00f4le\u017eit\u00fdmi faktormi, ktor\u00e9 vpl\u00fdvaj\u00fa na stav bud\u00facej matky, s\u00fa ot\u00e1zka genetiky, psychosoci\u00e1lne ot\u00e1zky, mo\u017en\u00e1 inkontinencia mo\u010du. \u010ealej ochorenie pe\u010dene alebo osteopor\u00f3za. Tak ako je samotn\u00e1 cystick\u00e1 fibr\u00f3za multiorg\u00e1nov\u00fdm ochoren\u00edm, je potrebn\u00e9 aj v \u010dase tehotenstva multidisciplin\u00e1rne mana\u017eova\u0165 jeho \u00faspe\u0161n\u00fd priebeh.<\/p>\n\n\n\n<p>Aj na Slovensku m\u00e1me zaznamenan\u00fdch nieko\u013eko \u00faspe\u0161n\u00fdch p\u00f4rodov CF matiek. Za v\u0161etky d\u00e1vam do pozornosti ned\u00e1vno medializovan\u00fd pr\u00edbeh <a href=\"https:\/\/ahojmama.pravda.sk\/clanky\/mamicka-s-cystickou-fibrozou-porodila-zdraveho-\/15326-clanok.html\">https:\/\/ahojmama.pravda.sk\/clanky\/mamicka-s-cystickou-fibrozou-porodila-zdraveho-\/15326-clanok.html<\/a><\/p>\n\n\n\n<h4 class=\"wp-block-heading\">Zhrnutie<\/h4>\n\n\n\n<p>So zvy\u0161uj\u00facim sa priemern\u00fdm vekom pacientiek s&nbsp;CF a ich&nbsp;t\u00fa\u017ebou sta\u0165 sa matkou, bude ot\u00e1zka tehotenstva \u010doraz \u010dastej\u0161ia. Je ve\u013emi d\u00f4le\u017eit\u00e9, aby sa pred t\u00fdmto krokom poradili s&nbsp;CF lek\u00e1rom, ktor\u00e9ho \u00falohou je informova\u0165 pacientku o&nbsp;mo\u017enom vplyve tehotenstva na jej zdravotn\u00fd stav a&nbsp;vplyve na tehotenstvo a&nbsp;plod. Po\u010das pr\u00edpravy na tehotenstvo sa optimalizuje v\u00fd\u017eiva, funkcie p\u013e\u00fac a&nbsp;fyzick\u00e1 zdatnos\u0165. &nbsp;Z\u00e1rove\u0148 sa mus\u00ed venova\u0165 pozornos\u0165 prehodnoteniu u\u017e\u00edvan\u00fdch liekov, CFRD, ochoreniu pe\u010dene, nedostatku vitam\u00ednov a&nbsp;pod. \u00daspe\u0161n\u00fdm p\u00f4rodom sa v\u0161ak starostlivos\u0165 o&nbsp;matku nekon\u010d\u00ed. Matku treba motivova\u0165 a&nbsp;povzbudzova\u0165, aby svoju lie\u010dbu po tehotenstve nezanedb\u00e1vala a&nbsp;venovala sa jej rovnako d\u00f4kladne ako pred tehotenstvom.<\/p>\n\n\n\n<p class=\"has-small-font-size\"><em>Zdroj:<\/em><\/p>\n\n\n\n<p class=\"has-small-font-size\"><em>[1] P. G. Middleton: Pregnancy in women with Cystic Fibrosis in the 21st century, Journal of Cystic Fibrosis 20 (2021)<\/em><br><em>[2]\u00a0 R. Oxman et al.: Gestational and pregestational diabetes in pregnant women with cystic fibrosis, Journal of Clinical &amp; Translational Endocrinology 27 (2022)<br>[3] E. F. Nash et al.: Outcomes of pregnancy in women with cystic fibrosis (CF) taking CFTR modulators \u2013 an international survey, Journal of Cystic Fibrosis 19 (2020)<br>[4] R. Jain et al.: Pregnancy in cystic fibrosis: Review of the literature and expert recommendations, Journal of Cystic Fibrosis<\/em><\/p>\n","protected":false},"excerpt":{"rendered":"<p>Lie&#269;ba cystickej fibr&oacute;zy (CF) sa za posledn&yacute;ch 50 rokov dramaticky zmenila v&#271;aka skr&iacute;ningu, skor&eacute;mu zavedeniu &uacute;&#269;innej lie&#269;by respira&#269;n&yacute;ch infekci&iacute; a pr&aacute;ci &scaron;pecializovan&yacute;ch centier. Skr&iacute;ning umo&#382;&#328;uje v&#269;asn&uacute; intervenciu v pr&iacute;pade &#271;al&scaron;&iacute;ch komplik&aacute;ci&iacute; vr&aacute;tane cukrovky, ochorenia pe&#269;ene a osteopor&oacute;zy. Dnes je be&#382;n&eacute;, &hellip; <a href=\"https:\/\/slanedeti.sk\/cms\/tehotenstvo-a-cysticka-fibroza\/2171\">Continued<\/a><\/p>\n","protected":false},"author":3,"featured_media":2173,"comment_status":"closed","ping_status":"closed","sticky":false,"template":"","format":"standard","meta":{"kt_blocks_editor_width":"","footnotes":""},"categories":[250],"tags":[],"class_list":["post-2171","post","type-post","status-publish","format-standard","has-post-thumbnail","hentry","category-zivot-s-cystickou-fibrozou"],"yoast_head":"<!-- This site is optimized with the Yoast SEO plugin v27.5 - https:\/\/yoast.com\/product\/yoast-seo-wordpress\/ -->\n<title>Tehotenstvo a cystick\u00e1 fibr\u00f3za - Slan\u00e9 deti<\/title>\n<meta name=\"description\" content=\"Lie\u010dba cystickej fibr\u00f3zy v\u010faka skr\u00edningu a \u00fa\u010dinnej lie\u010dbe umo\u017e\u0148uje, \u017ee sa aj CF pacientky m\u00f4\u017eu sta\u0165 matkami.\" \/>\n<meta name=\"robots\" content=\"index, follow, max-snippet:-1, max-image-preview:large, max-video-preview:-1\" \/>\n<link rel=\"canonical\" href=\"https:\/\/slanedeti.sk\/cms\/tehotenstvo-a-cysticka-fibroza\/2171\" \/>\n<meta property=\"og:locale\" content=\"sk_SK\" \/>\n<meta property=\"og:type\" content=\"article\" \/>\n<meta property=\"og:title\" content=\"Tehotenstvo a cystick\u00e1 fibr\u00f3za - 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