{"id":62438,"date":"2020-06-02T09:49:46","date_gmt":"2020-06-02T09:49:46","guid":{"rendered":"https:\/\/www.humanitas.net\/?post_type=disease&#038;p=62438"},"modified":"2020-11-03T13:37:15","modified_gmt":"2020-11-03T13:37:15","slug":"marfanov-sindrom","status":"publish","type":"disease","link":"https:\/\/www.humanitas.net\/sl\/diseases\/marfanov-sindrom\/","title":{"rendered":"Marfanov sindrom"},"content":{"rendered":"<h2>Opredelitev<\/h2>\n<p>Marfanov sindrom je dedna motnja. Vpliva na vezivno tkivo ali to so tkivna vlakna, ki povezujejo, podpirajo, ve\u017eejo ali lo\u010dujejo organe in druge strukture v telesu. Ta sindrom obi\u010dajno prizadene o\u010di, srce, o\u017eilje in okostje. Ljudje, ki imajo Marfanov sindrom, imajo nesorazmerno telo. So visoki in tanki, roke, prsti, noge in no\u017eni prsti so dalj\u0161i, kot bi morali biti obi\u010dajno v sorazmerju s preostankom telesa. \u0160koda, ki jo povzro\u010da Marfanov sindrom, je lahko blaga ali huda. Stanje je lahko resno, \u010de prizadene srce ali o\u017eilje. Vendar pa obi\u010dajno obstajajo zdravila, ki lahko stanje nadzirajo do dolo\u010dene to\u010dke. Morda bo potreben operativni poseg, odvisno od tega, kateri del telesa je prizadet in resnosti simptomov.<\/p>\n<p>&nbsp;<\/p>\n<h2>Simptomi<\/h2>\n<p>Simptomi Marfanovega sindroma so lahko razli\u010dni. Razlikujejo se lahko celo med \u010dlani ene dru\u017eine. Nekdo bo ob\u010dutil blage u\u010dinke, drugi pa lahko razvijejo nevarne zaplete. Na splo\u0161no se stanje s starostjo poslab\u0161a.<\/p>\n<p>Simptomi so lahko:<\/p>\n<ul>\n<li>Vitka zgradba<\/li>\n<li>Visoko in tanko telo<\/li>\n<li>Neproporcionalne roke, noge, prsti in prsti<\/li>\n<li>Ravna stopala<\/li>\n<li>Visoko obokano nepce in natrpani zobje<\/li>\n<li>Izjemna bli\u017eina<\/li>\n<li>Nenormalno ukrivljena hrbtenica<\/li>\n<\/ul>\n<p>&nbsp;<\/p>\n<h2>Vzroki<\/h2>\n<p>Marfanov sindrom se pojavi, kadar je gen okvarjen, ki telesu omogo\u010da proizvodnjo beljakovin, ki vezivnemu tkivu daje elasti\u010dnost in mo\u010d.<\/p>\n<p>Marfanov sindrom je na splo\u0161no podedovan. Obi\u010dajno ve\u010dina ljudi podeduje nenormalni gen od star\u0161ev, ki imajo motnjo. Otroci imajo 50-50% mo\u017enosti, da dedujejo pokvarjen gen od star\u0161ev. V 25% primerov se Marfanov sindrom ne podeduje, ampak pride do spontane mutacije gena.<\/p>\n<p>&nbsp;<\/p>\n<p>&nbsp;<\/p>\n<h2>Dejavniki tveganja<\/h2>\n<p>Marfanov sindrom lahko prizadene vsakogar, ne glede na spol, raso ali narodnost. Ker gre za gensko motnjo, je tveganje ve\u010dje, \u010de v dru\u017eini obstaja stanje, tj. \u010ce ima otrok star\u0161a z motnjo.<\/p>\n<p>&nbsp;<\/p>\n<h2>Zapleti<\/h2>\n<p>Marfanov sindrom lahko prizadene \u0161tevilne dele telesa, kot so: o\u010di, srce, o\u017eilje in okostje. To pomeni, da lahko povzro\u010di razli\u010dne zaplete.<\/p>\n<p>Kardiovaskularni zapleti, ki se lahko pojavijo, so:<\/p>\n<ul>\n<li>Anevrizma aorte<\/li>\n<li>Aortna disekcija<\/li>\n<li>Malformacije ventilov<\/li>\n<li>O\u010desni zapleti so lahko:<\/li>\n<li>Dislokacija objektiva<\/li>\n<li>Te\u017eave z mre\u017enico<\/li>\n<li>Glavokom ali katarakta zgodaj zgodaj<\/li>\n<li>Skeletni zapleti:<\/li>\n<li>Nenormalno ukrivljena hrbtenica<\/li>\n<li>Skolioza<\/li>\n<li>Potopljena prsna kost<\/li>\n<li>Bole\u010dine v stopalu<\/li>\n<li>Bole\u010dine v kri\u017eu<\/li>\n<\/ul>\n<p>Zapleti, ki se lahko pojavijo med nose\u010dnostjo &#8211; \u017eensko srce med nose\u010dnostjo \u010drpa ve\u010d krvi. \u010ce \u017eenska ima Marfanov sindrom, sindrom prizadene glavno arterijo, ki zapusti srce &#8211; aorto. Stene aorte lahko postanejo \u0161ibkej\u0161e in tako aorti doda dodaten stres, kar pove\u010da tveganje za smrtno disekcijo ali rupturo.<\/p>\n<h2>Zdravljenje<\/h2>\n<p>Ker zdravljenja za Marfanov sindrom ni, bo poudarek na zdravljenju prepre\u010devanje mo\u017enih zapletov. V preteklosti je bila \u017eivljenjska doba ljudi z Marfanovim sindromom zelo kratka, do 40 let. Dandanes zaradi sodobnih na\u010dinov zdravljenja in rednega spremljanja lahko ljudje pri\u010dakujejo bolj normalno \u017eivljenjsko dobo.<\/p>\n<p>Obstaja ve\u010d vrst zdravljenja, ki jih je mogo\u010de upo\u0161tevati. Zdravniki lahko: predpi\u0161ejo zdravila, kot so zdravila, ki zni\u017eujejo krvni tlak; priporo\u010damo no\u0161enje o\u010dal, kontaktnih le\u010d ali operacijo popravljanja dislocirane le\u010de v o\u010deh; priporo\u010damo kirur\u0161ke in druge postopke, odvisno od znakov, ki se pojavijo (nekateri primeri so: popravilo aorte, zdravljenje skolioze, korekcija prsne kosti in drugi).<\/p>\n<p>Priporo\u010dljivo je, da se nekaterim rekreacijskim dejavnostim in tekmovalnim \u0161portom izognemo, \u0161e posebej, \u010de ima oseba ve\u010dje tveganje za disecijo ali razpad aorte. Varnej\u0161i \u0161porti ali aktivnosti so tiste, ki niso zelo intenzivne, na primer: hitra hoja, dvojni tenis, golf ali balinanje.<\/p>\n<p>&nbsp;<\/p>\n","protected":false},"author":11,"featured_media":0,"template":"","yoast_head":"<!-- This site is optimized with the Yoast SEO plugin v19.7.1 - https:\/\/yoast.com\/wordpress\/plugins\/seo\/ -->\n<title>Marfanov sindrom - Humanitas.net<\/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:\/\/www.humanitas.net\/sl\/diseases\/marfanov-sindrom\/\" \/>\n<meta property=\"og:locale\" content=\"sl_SI\" \/>\n<meta property=\"og:type\" content=\"article\" \/>\n<meta property=\"og:title\" content=\"Marfanov sindrom - Humanitas.net\" \/>\n<meta property=\"og:description\" content=\"Opredelitev Marfanov sindrom je dedna motnja. 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Vpliva na vezivno tkivo ali to so tkivna vlakna, ki povezujejo, podpirajo, ve\u017eejo ali lo\u010dujejo organe in druge strukture v telesu. Ta sindrom obi\u010dajno prizadene o\u010di, srce, o\u017eilje in okostje. Ljudje, ki imajo Marfanov sindrom, imajo nesorazmerno telo. 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