{"id":1532,"date":"2020-02-18T20:56:36","date_gmt":"2020-02-18T19:56:36","guid":{"rendered":"http:\/\/klubcf.sk\/?page_id=1532"},"modified":"2020-02-18T20:58:43","modified_gmt":"2020-02-18T19:58:43","slug":"orkambi-liecba-znizuje-pecenove-problemy-stukovatenie-pecene-u-pacientov-s-cystickou-fibrozou","status":"publish","type":"page","link":"https:\/\/klubcf.sk\/index.php\/cysticka-fibroza\/orkambi-liecba-znizuje-pecenove-problemy-stukovatenie-pecene-u-pacientov-s-cystickou-fibrozou\/","title":{"rendered":"Orkambi lie\u010dba zni\u017euje pe\u010de\u0148ov\u00e9 probl\u00e9my (stukovatenie pe\u010dene) u\u00a0pacientov s\u00a0cystickou fibr\u00f3zou."},"content":{"rendered":"<p align=\"justify\"><span style=\"color: #444950;\"><span style=\"font-family: Arial, serif;\">Pacienti s CF, ktor\u00ed sa lie\u010dili s Orkambi (lumakaftor \/ ivakaftor) maj\u00fa v\u00fdznamne men\u0161ie nahromadenie tuku v pe\u010deni &#8211; stav zn\u00e1my ako steat\u00f3za -stukovatenie pe\u010dene &#8211; \u010do je \u010dasto spojen\u00e9 s CF. \u0160t\u00fadia -Lumakaftor\/ivakaftor bola zverejnen\u00e1 v \u010dasopise- The World Journal of Hepatology. Pacienti s CF m\u00f4\u017eu trpie\u0165 ochoren\u00edm pe\u010dene- s\u00favisiacim s ochoren\u00edm- (CFLD) a cukrovkou s\u00favisiacou s CF (CFRD). Najbe\u017enej\u0161ou formou CFLD je tzv. steat\u00f3za pe\u010dene &#8211; hromadenie tuku v pe\u010deni, \u010do m\u00f4\u017ee vies\u0165 k po\u0161kodeniu pe\u010dene. Vyskytuje sa a\u017e u 60% pacientov s CF a m\u00f4\u017ee prejs\u0165 k cirh\u00f3ze, \u010do je stav, ktor\u00fd si zvy\u010dajne vy\u017eaduje lie\u010dbu ako transplant\u00e1ciu pe\u010dene. CFRD (cukrovka s\u00favisiaca s CF) sa vyskytuje pribli\u017ene u 20% adolescentov s CF a a\u017e u 50% dospel\u00fdch. Vyzna\u010duje sa n\u00edzkym obsahom inzul\u00ednu alebo nedostato\u010dnos\u0165ou inzul\u00ednu. Je tu rozdiel, neprodukuje sa \u017eiadny inzul\u00edn, ako je to pri cukrovke typu 1, alebo inzul\u00ednov\u00e1 rezistencia, ktor\u00e1 sa pozoruje u pacientov s cukrovkou 2. typu. Pacienti, ktor\u00ed maj\u00fa mut\u00e1ciu F508del, m\u00f4\u017eu by\u0165 lie\u010den\u00ed liekom Orkambi. Formu tabletiek vyvinula spolo\u010dnos\u0165 Vertex Pharmaceuticals. Lie\u010dba sa za\u010d\u00edna od 2 rokov a vy\u0161\u0161ie.<\/span><\/span><\/p>\n<p align=\"justify\"><span style=\"color: #444950;\"><span style=\"font-family: Arial, serif;\">Orkambi, modul\u00e1tor g\u00e9nu CFTR , je kombin\u00e1ciu 2 zlo\u017eiek- lumakaftora, ktor\u00fd zvy\u0161uje po\u010det chloridov\u00fdch kan\u00e1likov a&nbsp;ivakaftora, ktor\u00fd je na trhu zn\u00e1my ako Kalydeko a pom\u00e1ha po\u0161koden\u00fdm kan\u00e1likom lep\u0161ie fungova\u0165.<\/span><\/span><\/p>\n<p align=\"justify\"><span style=\"color: #444950;\"><span style=\"font-family: Arial, serif;\">CFTR modul\u00e1tory, ako Orkambi, zlep\u0161ili funkciu p\u013e\u00fac pacientov s&nbsp;CF, \u010do viedlo k&nbsp;ni\u017e\u0161iemu po\u010dtu hospitaliz\u00e1ci\u00ed a&nbsp;menej \u010dastej potrebe u\u017e\u00edva\u0165 antibiotik\u00e1. Doposia\u013e \u017eiadna \u0161t\u00fadia nepoukazovala na vplyv modul\u00e1torov na steat\u00f3zu.<\/span><\/span><\/p>\n<p align=\"justify\"><span style=\"color: #444950;\"><span style=\"font-family: Arial, serif;\">T\u00edm v\u00fdskumn\u00edkov z&nbsp;\u201eCase Western Reserve University\u201c v&nbsp;Ohiu sa rozhodol presk\u00fama\u0165 roz\u0161\u00edrenie hepatickej steat\u00f3zy medzi pacientmi s&nbsp;CF , rovnako medzi t\u00fdmi, ktor\u00fd s\u00fa na lie\u010dbe Orkambi ako aj medzi pacientami, ktor\u00ed Orkambi nedost\u00e1vaj\u00fa. T\u00edm taktie\u017e sk\u00famal prepojenie cukrovky s\u00favisiacej s&nbsp;CF (CFRD) a&nbsp;hepatickej steat\u00f3zy , ke\u010f\u017ee je \u00fazko spojen\u00e1 s&nbsp;obezitou a&nbsp;cukrovkou 2.typu u&nbsp;pacientov, ktor\u00ed netrpia CF.<\/span><\/span><\/p>\n<p align=\"justify\"><span style=\"color: #444950;\"><span style=\"font-family: Arial, serif;\">\u0160t\u00fadie sa z\u00fa\u010dastnilo 20 pacientov vo veku 11 a\u017e 39 rokov. 12 z&nbsp;nich malo cukrovku CFRD spojen\u00fa s&nbsp;CF a&nbsp;8 pacientov malo norm\u00e1lnu hladinu cukru. 7 pacientov z&nbsp;t\u00fdch, ktor\u00ed trpia CFRD boli lie\u010den\u00ed s&nbsp;Orkambi, rovnako aj 2 pacienti bez CFRD.<\/span><\/span><\/p>\n<p align=\"justify\"><span style=\"color: #444950;\"><span style=\"font-family: Arial, serif;\">Objem tuku v&nbsp;pe\u010deni bol meran\u00fd magnetickou rezonanciou MRI pomocou stanovenia tukovej frakcie prot\u00f3novej hustoty (PDFF). PDFF nad 5% bola vyhodnoten\u00e1 ako klinick\u00e1 hepatick\u00e1 steat\u00f3za.<\/span><\/span><\/p>\n<p align=\"justify\"><span style=\"color: #444950;\"><span style=\"font-family: Arial, serif;\">V\u00fdsledky v\u00fdskumu uk\u00e1zali rozsah PDFF od 0% do 21%. 6 pacientov z\u00fa\u010dastnen\u00fdch na \u0161t\u00fadi\u00ed (30%) malo hepatick\u00fa steat\u00f3zu.<\/span><\/span><\/p>\n<p align=\"justify\"><span style=\"color: #444950;\"><span style=\"font-family: Arial, serif;\">V\u0161etk\u00fdch 9 pacientov lie\u010den\u00fdch Orkambi malo ove\u013ea ni\u017e\u0161ie hodnoty PDFF, v&nbsp;rozsahu 0% a\u017e 6,4%, s&nbsp;priemerom 2%, v&nbsp;porovnan\u00ed s&nbsp;t\u00fdmi pacientmi, ktor\u00ed neboli lie\u010den\u00ed modul\u00e1tormi. T\u00ed pacienti, ktor\u00ed neboli lie\u010den\u00ed s&nbsp;Orkambi, dosahovali priemern\u00fa hodnotu 4,1%, s&nbsp;rozsahom od 2,7% do 21%.<\/span><\/span><\/p>\n<p align=\"justify\"><span style=\"color: #444950;\"><span style=\"font-family: Arial, serif;\">\u010co sa t\u00fdka potenci\u00e1lneho prepojenia cukrovky spojenej s&nbsp;CF (CFRD) a&nbsp;hepatickej steat\u00f3zy, v\u00fdsledky uk\u00e1zali, \u017ee pacienti trpiaci CFRD mali ni\u017e\u0161ie nameran\u00e9 hodnoty PDFF v&nbsp;porovnan\u00ed s&nbsp;pacientmi bez cukrovky. Napriek tomu, \u017ee rozdiel nebol \u0161tatisticky v\u00fdznamn\u00fd, m\u00f4\u017eeme poveda\u0165, \u017ee nie je \u017eiadna s\u00favislos\u0165 cukrovky CFRD a&nbsp;hepatickej steat\u00f3zy.<\/span><\/span><\/p>\n<p align=\"justify\"><span style=\"color: #444950;\"><span style=\"font-family: Arial, serif;\">Z\u00e1ver: <\/span><\/span><span style=\"color: #444950;\"><span style=\"font-family: Arial, serif;\"><b>\u0160t\u00fadia dok\u00e1zala ve\u013ek\u00fa s\u00favislos\u0165 u\u017e\u00edvania lumakaftorov a&nbsp;ivakaftorov (Orkambi ) a&nbsp;zn\u00ed\u017eenia hepatickej steat\u00f3zy u&nbsp;pacientov s&nbsp;CF.<\/b><\/span><\/span><\/p>\n<p align=\"justify\"><span style=\"color: #444950;\"><span style=\"font-family: Arial, serif;\">Zdroj: <\/span><\/span><a href=\"https:\/\/cysticfibrosisnewstoday.com\/2020\/02\/11\/orkambi-therapy-reduces-hepatic-steatosis-cf-patients-study-shows\/?fbclid=IwAR2GYZvEC6Mf5LY2gQTIA6gjmEARPNsnuNRh9scBiWrmyPGALCJq_ga9aPo\">https:\/\/cysticfibrosisnewstoday.com\/2020\/02\/11\/orkambi-therapy-reduces-hepatic-steatosis-cf-patients-study-shows\/?fbclid=IwAR2GYZvEC6Mf5LY2gQTIA6gjmEARPNsnuNRh9scBiWrmyPGALCJq_ga9aPo<\/a>&nbsp;<\/p>\n<p align=\"justify\"><img loading=\"lazy\" decoding=\"async\" class=\"alignnone size-full wp-image-1536\" src=\"http:\/\/klubcf.sk\/wp-content\/uploads\/2020\/02\/pe\u010de\u0148-1.jpg\" alt=\"\" width=\"720\" height=\"481\" srcset=\"https:\/\/klubcf.sk\/wp-content\/uploads\/2020\/02\/pe\u010de\u0148-1.jpg 720w, https:\/\/klubcf.sk\/wp-content\/uploads\/2020\/02\/pe\u010de\u0148-1-300x200.jpg 300w, https:\/\/klubcf.sk\/wp-content\/uploads\/2020\/02\/pe\u010de\u0148-1-570x381.jpg 570w\" sizes=\"auto, (max-width: 720px) 100vw, 720px\" \/><\/p>\n","protected":false},"excerpt":{"rendered":"<p>Pacienti s CF, ktor\u00ed sa lie\u010dili s Orkambi (lumakaftor \/ ivakaftor) maj\u00fa v\u00fdznamne men\u0161ie nahromadenie tuku v pe\u010deni &#8211; stav zn\u00e1my ako steat\u00f3za -stukovatenie pe\u010dene &#8211; \u010do je \u010dasto spojen\u00e9 s CF. \u0160t\u00fadia -Lumakaftor\/ivakaftor bola zverejnen\u00e1 v \u010dasopise- The World Journal of Hepatology. Pacienti s CF m\u00f4\u017eu trpie\u0165 ochoren\u00edm pe\u010dene- s\u00favisiacim s ochoren\u00edm- (CFLD) a [&hellip;]<\/p>\n","protected":false},"author":1,"featured_media":1536,"parent":29,"menu_order":0,"comment_status":"closed","ping_status":"closed","template":"","meta":{"ngg_post_thumbnail":0,"footnotes":""},"class_list":["post-1532","page","type-page","status-publish","has-post-thumbnail","hentry"],"yoast_head":"<!-- This site is optimized with the Yoast SEO plugin v27.2 - https:\/\/yoast.com\/product\/yoast-seo-wordpress\/ -->\n<title>Orkambi lie\u010dba zni\u017euje pe\u010de\u0148ov\u00e9 probl\u00e9my (stukovatenie pe\u010dene) u\u00a0pacientov s\u00a0cystickou fibr\u00f3zou. - klubcf.sk<\/title>\n<meta name=\"robots\" content=\"index, follow, max-snippet:-1, max-image-preview:large, max-video-preview:-1\" \/>\n<link rel=\"canonical\" href=\"https:\/\/klubcf.sk\/index.php\/cysticka-fibroza\/orkambi-liecba-znizuje-pecenove-problemy-stukovatenie-pecene-u-pacientov-s-cystickou-fibrozou\/\" \/>\n<meta property=\"og:locale\" content=\"sk_SK\" \/>\n<meta property=\"og:type\" content=\"article\" \/>\n<meta property=\"og:title\" content=\"Orkambi lie\u010dba zni\u017euje pe\u010de\u0148ov\u00e9 probl\u00e9my (stukovatenie pe\u010dene) u\u00a0pacientov s\u00a0cystickou fibr\u00f3zou. - klubcf.sk\" \/>\n<meta property=\"og:description\" content=\"Pacienti s CF, ktor\u00ed sa lie\u010dili s Orkambi (lumakaftor \/ ivakaftor) maj\u00fa v\u00fdznamne men\u0161ie nahromadenie tuku v pe\u010deni &#8211; stav zn\u00e1my ako steat\u00f3za -stukovatenie pe\u010dene &#8211; \u010do je \u010dasto spojen\u00e9 s CF. \u0160t\u00fadia -Lumakaftor\/ivakaftor bola zverejnen\u00e1 v \u010dasopise- The World Journal of Hepatology. Pacienti s CF m\u00f4\u017eu trpie\u0165 ochoren\u00edm pe\u010dene- s\u00favisiacim s ochoren\u00edm- (CFLD) a [&hellip;]\" \/>\n<meta property=\"og:url\" content=\"https:\/\/klubcf.sk\/index.php\/cysticka-fibroza\/orkambi-liecba-znizuje-pecenove-problemy-stukovatenie-pecene-u-pacientov-s-cystickou-fibrozou\/\" \/>\n<meta property=\"og:site_name\" content=\"klubcf.sk\" \/>\n<meta property=\"article:modified_time\" content=\"2020-02-18T19:58:43+00:00\" \/>\n<meta property=\"og:image\" content=\"https:\/\/klubcf.sk\/wp-content\/uploads\/2020\/02\/pe\u010de\u0148-1.jpg\" \/>\n\t<meta property=\"og:image:width\" content=\"720\" \/>\n\t<meta property=\"og:image:height\" content=\"481\" \/>\n\t<meta property=\"og:image:type\" content=\"image\/jpeg\" \/>\n<meta name=\"twitter:card\" content=\"summary_large_image\" \/>\n<meta name=\"twitter:label1\" content=\"Predpokladan\u00fd \u010das \u010d\u00edtania\" \/>\n\t<meta name=\"twitter:data1\" content=\"3 min\u00faty\" \/>\n<script type=\"application\/ld+json\" class=\"yoast-schema-graph\">{\"@context\":\"https:\/\/schema.org\",\"@graph\":[{\"@type\":\"WebPage\",\"@id\":\"https:\/\/klubcf.sk\/index.php\/cysticka-fibroza\/orkambi-liecba-znizuje-pecenove-problemy-stukovatenie-pecene-u-pacientov-s-cystickou-fibrozou\/\",\"url\":\"https:\/\/klubcf.sk\/index.php\/cysticka-fibroza\/orkambi-liecba-znizuje-pecenove-problemy-stukovatenie-pecene-u-pacientov-s-cystickou-fibrozou\/\",\"name\":\"Orkambi lie\u010dba zni\u017euje pe\u010de\u0148ov\u00e9 probl\u00e9my (stukovatenie pe\u010dene) u\u00a0pacientov s\u00a0cystickou fibr\u00f3zou. - 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