{"id":7891,"date":"2018-05-27T16:44:24","date_gmt":"2018-05-27T19:44:24","guid":{"rendered":"https:\/\/blog.mindthegraph.com\/?p=7891"},"modified":"2022-10-18T14:37:51","modified_gmt":"2022-10-18T17:37:51","slug":"cystic-fibrosis","status":"publish","type":"post","link":"https:\/\/mindthegraph.com\/blog\/hu\/cisztas-fibrozis\/","title":{"rendered":"A ciszt\u00e1s fibr\u00f3zisra val\u00f3 figyelemfelh\u00edv\u00e1s h\u00f3napja"},"content":{"rendered":"<p>M\u00e1jus a ciszt\u00e1s fibr\u00f3zisra val\u00f3 figyelemfelh\u00edv\u00e1s h\u00f3napja. A ciszt\u00e1s fibr\u00f3zis (CF) autoszom\u00e1lis recessz\u00edv genetikai rendelleness\u00e9g. A CF-et a ciszt\u00e1s fibr\u00f3zis transzmembr\u00e1n konduktancia szab\u00e1lyoz\u00f3 (CFTR) csatorn\u00e1t k\u00f3dol\u00f3 g\u00e9n mut\u00e1ci\u00f3i okozz\u00e1k.  Ez a feh\u00e9rje felel\u0151s a sejtmembr\u00e1nokon kereszt\u00fcli ionsz\u00e1ll\u00edt\u00e1s elt\u00e9r\u00e9s\u00e9\u00e9rt.<\/p>\n\n\n\n<p>Genetikai otthoni referenci\u00e1kkal \u00f6sszhangban <sup>1<\/sup>, a CFTR g\u00e9n a ciszt\u00e1s fibr\u00f3zis transzmembr\u00e1n konduktancia szab\u00e1lyoz\u00f3j\u00e1nak nevezett feh\u00e9rje el\u0151\u00e1ll\u00edt\u00e1s\u00e1hoz ad utas\u00edt\u00e1sokat:<\/p>\n\n\n\n<p>Ez a feh\u00e9rje csatornak\u00e9nt m\u0171k\u00f6dik a ny\u00e1lk\u00e1t, verejt\u00e9ket, ny\u00e1lat, k\u00f6nnyet \u00e9s em\u00e9szt\u0151enzimeket termel\u0151 sejtek membr\u00e1nj\u00e1n kereszt\u00fcl. A csatorna negat\u00edv t\u00f6lt\u00e9s\u0171 r\u00e9szecsk\u00e9ket, \u00fagynevezett kloridionokat sz\u00e1ll\u00edt a sejtekbe \u00e9s a sejtekb\u0151l. A kloridionok sz\u00e1ll\u00edt\u00e1sa seg\u00edt szab\u00e1lyozni a v\u00edz mozg\u00e1s\u00e1t a sz\u00f6vetekben, ami sz\u00fcks\u00e9ges a v\u00e9kony, szabadon \u00e1raml\u00f3 ny\u00e1lka termel\u00e9s\u00e9hez. A ny\u00e1lka egy cs\u00fasz\u00f3s anyag, amely ken\u0151dik \u00e9s v\u00e9di a l\u00e9gutak, az em\u00e9szt\u0151rendszer, a reprodukt\u00edv rendszer \u00e9s m\u00e1s szervek \u00e9s sz\u00f6vetek b\u00e9l\u00e9s\u00e9t.<\/p>\n\n\n\n<p><span style=\"font-weight: 400;\">A CFTR feh\u00e9rje m\u00e1s csatorn\u00e1k m\u0171k\u00f6d\u00e9s\u00e9t is szab\u00e1lyozza, p\u00e9ld\u00e1ul azok\u00e9t, amelyek pozit\u00edv t\u00f6lt\u00e9s\u0171 r\u00e9szecsk\u00e9ket, \u00fagynevezett n\u00e1triumionokat sz\u00e1ll\u00edtanak a sejtmembr\u00e1nokon kereszt\u00fcl. Ezek a csatorn\u00e1k sz\u00fcks\u00e9gesek az olyan szervek norm\u00e1lis m\u0171k\u00f6d\u00e9s\u00e9hez, mint a t\u00fcd\u0151 \u00e9s a hasny\u00e1lmirigy.<\/span><\/p>\n\n\n<div class=\"wp-block-image\">\n<figure class=\"aligncenter\"><a href=\"https:\/\/mindthegraph.com\/science_figures\" target=\"_blank\" rel=\"https:\/\/mindthegraph.com\/science_figures noopener\"><img decoding=\"async\" loading=\"lazy\" width=\"424\" height=\"306\" src=\"https:\/\/blog.mindthegraph.com\/wp-content\/uploads\/2018\/05\/dfaefqawefafc.png\" alt=\"dfaefqawefafc\" class=\"wp-image-7917\" srcset=\"https:\/\/mindthegraph.com\/blog\/wp-content\/uploads\/2018\/05\/dfaefqawefafc.png 424w, https:\/\/mindthegraph.com\/blog\/wp-content\/uploads\/2018\/05\/dfaefqawefafc-300x217.png 300w\" sizes=\"(max-width: 424px) 100vw, 424px\" \/><\/a><\/figure><\/div>\n\n\n<p>Ez befoly\u00e1solja az exokrin mirigyek m\u0171k\u00f6d\u00e9s\u00e9t, s\u0171r\u0171bb, nehezen elt\u00e1vol\u00edthat\u00f3 anyagokat (ny\u00e1lka, verejt\u00e9k vagy hasny\u00e1lmirigy-enzimek) termelve.<br>Emiatt a ciszt\u00e1s fibr\u00f3zis az em\u00e9szt\u0151rendszert, a l\u00e9gz\u0151szerveket \u00e9s a verejt\u00e9kmirigyeket \u00e9rinti.<\/p>\n\n\n\n<p>A betegs\u00e9g azonban legink\u00e1bb a l\u00e9gz\u0151rendszert \u00e9rinti. A t\u00fcd\u0151 s\u0171r\u0171 ny\u00e1lk\u00e1t termel, amely megrekedhet a l\u00e9gutakban. A ciszt\u00e1s fibr\u00f3zisban szenved\u0151 betegekn\u00e9l kr\u00f3nikus bakteri\u00e1lis t\u00fcd\u0151fert\u0151z\u00e9sek \u00e9s gyullad\u00e1sok alakulhatnak ki, amelyek v\u00e9g\u00fcl v\u00e9gst\u00e1dium\u00fa t\u00fcd\u0151betegs\u00e9get okozhatnak.<br>Tov\u00e1bbi t\u00fcnetek a v\u00e1lad\u00e9kos k\u00f6h\u00f6g\u00e9s, a visszat\u00e9r\u0151 t\u00fcd\u0151gyullad\u00e1s \u00e9s a kr\u00f3nikus h\u00f6rghurut.<\/p>\n\n\n\n<p>A CF-ben szenved\u0151 n\u0151k nehezebben esnek teherbe, mivel a vastagabb m\u00e9hnyakv\u00e1lad\u00e9k megnehez\u00edti a spermiumok \u00e1tjut\u00e1s\u00e1t. A f\u00e9rfiak sz\u00e1m\u00e1ra ez m\u00e9g nehezebb. A f\u00e9rfiak k\u00f6r\u00fclbel\u00fcl 98%-je medd\u0151, b\u00e1r teljesen norm\u00e1lis szexu\u00e1lis teljes\u00edt\u0151k\u00e9pess\u00e9ggel \u00e9s potenci\u00e1val rendelkeznek.<\/p>\n\n\n\n<p>Ma 3 CF linket v\u00e1lasztottam, hogy megosszam veletek.<\/p>\n\n\n\n<div class=\"is-content-justification-center is-layout-flex wp-container-1 wp-block-buttons\">\n<div class=\"wp-block-button\"><a class=\"wp-block-button__link\" href=\"https:\/\/mindthegraph.com\/science_figures\">Hat\u00e9kony tudom\u00e1nyos \u00e1br\u00e1k l\u00e9trehoz\u00e1sa percek alatt<\/a><\/div>\n<\/div>\n\n\n\n<h3>A j\u00f3 k\u00f6zlem\u00e9ny a ciszt\u00e1s fibr\u00f3zis demisztifik\u00e1l\u00e1sa<\/h3>\n\n\n\n<center>\n<p><\/p><\/center>\n\n\n<p><iframe loading=\"lazy\" title=\"Mi a ciszt\u00e1s fibr\u00f3zis?\" width=\"800\" height=\"450\" src=\"https:\/\/www.youtube.com\/embed\/k4U5wyPzxJE?feature=oembed\" frameborder=\"0\" allow=\"accelerometer; autoplay; clipboard-write; encrypted-media; gyroscope; picture-in-picture\" allowfullscreen><\/iframe><\/p>\n\n\n\n<h3>15 dolog, amit a ciszt\u00e1s fibr\u00f3zissal \u00e9l\u0151 emberek szeretn\u00e9nek, ha tudn\u00e1l - a buzzfeed list\u00e1ja<\/h3>\n\n\n\n<p>A CF-vel \u00e9lni nem k\u00f6nny\u0171, ez\u00e9rt nagyon fontos, hogy legyen t\u00e1mogat\u00e1s \u00e9s besz\u00e9lj\u00fcnk r\u00f3la. N\u00e9zd meg ezt a buzzfeed list\u00e1t<sup>2<\/sup> , hogy ciszt\u00e1s fibr\u00f3zisban szenved\u0151 emberek besz\u00e9ljenek a mindennapjaikr\u00f3l.<\/p>\n\n\n\n<div class=\"is-content-justification-center is-layout-flex wp-container-2 wp-block-buttons\">\n<div class=\"wp-block-button\"><a class=\"wp-block-button__link\" href=\"https:\/\/mindthegraph.com\/science_figures\">Kezdje el haszn\u00e1lni a Mind The Graph-ot most ingyen!<\/a><\/div>\n<\/div>\n\n\n\n<h3>&nbsp;Fogkr\u00e9mben tal\u00e1lhat\u00f3 antibakteri\u00e1lis vegy\u00fclet<sup>3<\/sup>, az \u00fagynevezett trikloz\u00e1n, r\u00e9sze lehet a j\u00f6v\u0151beni ciszt\u00e1s fibr\u00f3zis (CF) kezel\u00e9snek.<\/h3>\n\n\n<div class=\"wp-block-image size-full wp-image-7921\">\n<figure class=\"aligncenter\"><a href=\"https:\/\/mindthegraph.com\/science_figures\" target=\"_blank\" rel=\"https:\/\/mindthegraph.com\/science_figures noopener\"><img decoding=\"async\" loading=\"lazy\" width=\"800\" height=\"420\" src=\"https:\/\/blog.mindthegraph.com\/wp-content\/uploads\/2018\/05\/31846788804_1d47e5b32b_b_0-1.jpg\" alt=\"Ez a k\u00e9p a Pseudomonas aeruginosa egysejt\u0171 bakt\u00e9rium milli\u00f3ib\u00f3l \u00e1ll\u00f3 bonyolult kol\u00f3ni\u00e1t mutat.\" class=\"wp-image-7921\" srcset=\"https:\/\/mindthegraph.com\/blog\/wp-content\/uploads\/2018\/05\/31846788804_1d47e5b32b_b_0-1.jpg 800w, https:\/\/mindthegraph.com\/blog\/wp-content\/uploads\/2018\/05\/31846788804_1d47e5b32b_b_0-1-300x158.jpg 300w, https:\/\/mindthegraph.com\/blog\/wp-content\/uploads\/2018\/05\/31846788804_1d47e5b32b_b_0-1-768x403.jpg 768w\" sizes=\"(max-width: 800px) 100vw, 800px\" \/><\/a><figcaption>Ez a k\u00e9p a Pseudomonas aeruginosa egysejt\u0171 bakt\u00e9rium milli\u00f3ib\u00f3l \u00e1ll\u00f3 bonyolult kol\u00f3ni\u00e1t mutat.<\/figcaption><\/figure><\/div>\n\n\n<p>A kutat\u00e1s&nbsp;<strong><em>A trikloz\u00e1n aminoglikozid adjuv\u00e1ns a Pseudomonas aeruginosa biofilmek felsz\u00e1mol\u00e1s\u00e1ban <sup>4<\/sup>&nbsp;<\/em><\/strong>azt mutatj\u00e1k, hogy a kombin\u00e1ci\u00f3 n\u00f6veli a P. aeruginosa biofilmekben l\u00e9v\u0151 sejtek elpuszt\u00edt\u00e1s\u00e1nak m\u00e9rt\u00e9k\u00e9t \u00e9s ar\u00e1ny\u00e1t, ami fontos klinikai k\u00f6vetkezm\u00e9nyekkel j\u00e1r a CF-betegek biofilmfert\u0151z\u00e9seinek kezel\u00e9s\u00e9re.<\/p>\n\n\n\n<ol><li><a href=\"https:\/\/medlineplus.gov\/genetics\/gene\/cftr\/\" target=\"_blank\" rel=\"noreferrer noopener\">CFTR g\u00e9n ciszt\u00e1s fibr\u00f3zis transzmembr\u00e1n konduktancia szab\u00e1lyoz\u00f3.<\/a><\/li><li><a href=\"https:\/\/www.buzzfeed.com\/ailbhemalone\/living-with-cystic-fibrosis?utm_term=.srElmo7oO#.nyQVXbMb2\" target=\"_blank\" rel=\"noreferrer noopener\">15 dolog, amit a ciszt\u00e1s fibr\u00f3zissal \u00e9l\u0151 emberek szeretn\u00e9nek, ha tudn\u00e1l.<\/a><\/li><li><a href=\"https:\/\/cysticfibrosisnewstoday.com\/2018\/05\/21\/cystic-fibrosis-antiobiotic-more-effective-in-combo-with-toothpaste-antibacterial-study-suggests\/\" target=\"_blank\" rel=\"noreferrer noopener\">A fogkr\u00e9mben l\u00e9v\u0151 antibakteri\u00e1lis trikloz\u00e1n j\u00f3l m\u0171k\u00f6dik kombin\u00e1lt antibiotikumos kezel\u00e9sk\u00e9nt, a tanulm\u00e1ny szerint.<\/a><\/li><li><a href=\"https:\/\/journals.asm.org\/doi\/full\/10.1128\/AAC.00146-18\" target=\"_blank\" rel=\"noreferrer noopener\">A trikloz\u00e1n egy aminoglikozid adjuv\u00e1ns az eradik\u00e1ci\u00f3hoz a&nbsp;<em>Pseudomonas aeruginosa<\/em>&nbsp;Biofilmek.<\/a><\/li><\/ol>","protected":false},"excerpt":{"rendered":"<p>M\u00e1jus a ciszt\u00e1s fibr\u00f3zisra val\u00f3 figyelemfelh\u00edv\u00e1s h\u00f3napja. A ciszt\u00e1s fibr\u00f3zis (CF) autoszom\u00e1lis recessz\u00edv genetikai rendelleness\u00e9g. A CF-et a ciszt\u00e1s fibr\u00f3zis transzmembr\u00e1n konduktancia szab\u00e1lyoz\u00f3 (CFTR) csatorn\u00e1t k\u00f3dol\u00f3 g\u00e9n mut\u00e1ci\u00f3i okozz\u00e1k.  Ez a feh\u00e9rje felel\u0151s a sejtmembr\u00e1nokon kereszt\u00fcli ionsz\u00e1ll\u00edt\u00e1s elt\u00e9r\u00e9s\u00e9\u00e9rt. A Genetics home referenci\u00e1k 1 szerint a CFTR g\u00e9n biztos\u00edtja a [...]<\/p>","protected":false},"author":11,"featured_media":7919,"comment_status":"open","ping_status":"open","sticky":false,"template":"","format":"image","meta":[],"categories":[959,28],"tags":[554,250],"yoast_head":"<!-- This site is optimized with the Yoast SEO plugin v19.9 - https:\/\/yoast.com\/wordpress\/plugins\/seo\/ -->\n<title>Cystic fibrosis awareness month - Mind the Graph Blog<\/title>\n<meta name=\"description\" content=\"May is the cystic fibrosis awareness month. Cystic fibrosis (CF) is an autosomal recessive genetic disorder. I chose 3 links of CF to share with you today:\" \/>\n<meta name=\"robots\" content=\"index, follow, max-snippet:-1, max-image-preview:large, max-video-preview:-1\" \/>\n<link rel=\"canonical\" href=\"https:\/\/mindthegraph.com\/blog\/hu\/cisztas-fibrozis\/\" \/>\n<meta property=\"og:locale\" content=\"hu_HU\" \/>\n<meta property=\"og:type\" content=\"article\" \/>\n<meta property=\"og:title\" content=\"Cystic fibrosis awareness month - Mind the Graph Blog\" \/>\n<meta property=\"og:description\" content=\"May is the cystic fibrosis awareness month. Cystic fibrosis (CF) is an autosomal recessive genetic disorder. I chose 3 links of CF to share with you today:\" \/>\n<meta property=\"og:url\" content=\"https:\/\/mindthegraph.com\/blog\/hu\/cisztas-fibrozis\/\" \/>\n<meta property=\"og:site_name\" content=\"Mind the Graph Blog\" \/>\n<meta property=\"article:published_time\" content=\"2018-05-27T19:44:24+00:00\" \/>\n<meta property=\"article:modified_time\" content=\"2022-10-18T17:37:51+00:00\" \/>\n<meta property=\"og:image\" content=\"https:\/\/mindthegraph.com\/blog\/wp-content\/uploads\/2018\/05\/31846788804_1d47e5b32b_b_0.jpg\" \/>\n\t<meta property=\"og:image:width\" content=\"800\" \/>\n\t<meta property=\"og:image:height\" content=\"420\" \/>\n\t<meta property=\"og:image:type\" content=\"image\/jpeg\" \/>\n<meta name=\"author\" content=\"Fabiola Soares\" \/>\n<meta name=\"twitter:card\" content=\"summary_large_image\" \/>\n<meta name=\"twitter:label1\" content=\"Written by\" \/>\n\t<meta name=\"twitter:data1\" content=\"Fabiola Soares\" \/>\n\t<meta name=\"twitter:label2\" content=\"Est. reading time\" \/>\n\t<meta name=\"twitter:data2\" content=\"3 minutes\" \/>\n<!-- \/ Yoast SEO plugin. -->","yoast_head_json":{"title":"Cystic fibrosis awareness month - Mind the Graph Blog","description":"May is the cystic fibrosis awareness month. Cystic fibrosis (CF) is an autosomal recessive genetic disorder. I chose 3 links of CF to share with you today:","robots":{"index":"index","follow":"follow","max-snippet":"max-snippet:-1","max-image-preview":"max-image-preview:large","max-video-preview":"max-video-preview:-1"},"canonical":"https:\/\/mindthegraph.com\/blog\/hu\/cisztas-fibrozis\/","og_locale":"hu_HU","og_type":"article","og_title":"Cystic fibrosis awareness month - Mind the Graph Blog","og_description":"May is the cystic fibrosis awareness month. Cystic fibrosis (CF) is an autosomal recessive genetic disorder. 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