{"id":29188,"date":"2017-09-29T18:26:23","date_gmt":"2017-09-29T16:26:23","guid":{"rendered":"http:\/\/www.invarena.cz\/?p=29188"},"modified":"2017-09-29T19:44:08","modified_gmt":"2017-09-29T17:44:08","slug":"le-figaro-za-legendami-o-upirech-stoji-mozna-nemoc-krve","status":"publish","type":"post","link":"https:\/\/www.invarena.cz\/?p=29188","title":{"rendered":"Le Figaro: Za legendami o up\u00edrech stoj\u00ed mo\u017en\u00e1 nemoc krve"},"content":{"rendered":"

Pot\u0159ebuje ke sv\u00e9mu \u017eivotu lidskou krev, vych\u00e1z\u00ed pouze v noci a st\u0159e\u017e\u00ed se sv\u011btla: to jsou n\u011bkter\u00e9 znaky vamp\u00fdra \u010dili up\u00edra. Podle v\u011bdc\u016f v\u0161ak tyto p\u0159\u00edznaky zp\u016fsobuje skute\u010dn\u011b existuj\u00edc\u00ed nemoc, erytropoetick\u00e1 protoporfyrie (EPP). Francouzsko-americk\u00fd t\u00fdm identifikoval genetickou mutaci, kter\u00e1 tuto chorobu vyvol\u00e1v\u00e1. Bledost, pot\u0159eba krve a citlivost na sv\u011btlo, t\u00edm v\u0161\u00edm se vyzna\u010duje, p\u00ed\u0161e francouzsk\u00fd den\u00edk Le Figaro.<\/strong><\/p>\n

Porfyrie tvo\u0159\u00ed skupinu osmi d\u011bdi\u010dn\u00fdch onemocn\u011bn\u00ed krve, kter\u00e1 se projevuj\u00ed v r\u016fzn\u00fdch form\u00e1ch. Spole\u010dn\u00e9 jim je to, \u017ee p\u0159i nich chyb\u00ed proces produkce hemu, kter\u00fd umo\u017e\u0148uje v\u00e1zat \u017eelezo v hemoglobinu, proteinu, kter\u00fd p\u0159en\u00e1\u0161\u00ed kysl\u00edk. Barvu krvi dod\u00e1v\u00e1 pr\u00e1v\u011b vazba mezi hemem a \u017eelezem.<\/p>\n

Za m\u00fdty o up\u00edrech m\u016f\u017ee st\u00e1t jedna z t\u011bchto porfyri\u00ed: erytropoetick\u00e1 protoporfyrie. Jde o nejb\u011b\u017en\u011bj\u0161\u00ed porfyrii, kter\u00e1 za\u010d\u00edn\u00e1 v d\u011btstv\u00ed. „Osoby posti\u017een\u00e9 erytropoetickou protoporfyri\u00ed trp\u00ed chronickou anemi\u00ed, kter\u00e1 zp\u016fsobuje, \u017ee jsou velmi bled\u00e9 a velmi unaven\u00e9,“ uv\u00e1d\u00ed doktor Barry Paw, jeden z hlavn\u00edch autor\u016f studie.<\/p>\n

Poko\u017eka nemocn\u00fdch se st\u00e1v\u00e1 velmi citlivou na sv\u011btlo. Sice je nezm\u011bn\u00ed v hrom\u00e1dku popela, ale p\u0159esto i pod mra\u010dn\u00fdm nebem je dosti slune\u010dn\u00edho UV z\u00e1\u0159en\u00ed na to, aby vyvolalo u nemocn\u00fdch vznik puch\u00fd\u0159\u016f a znetvo\u0159en\u00ed nosu nebo u\u0161n\u00edch boltc\u016f.<\/p>\n

A jak\u00e1 jsou doporu\u010den\u00ed pro ty, kdo erytropoetickou protoporfyri\u00ed trp\u00ed? Nevych\u00e1zet p\u0159es den a dost\u00e1vat transfuze krve bohat\u00e9 na hem, aby se omezily p\u0159\u00edznaky choroby. Je mo\u017en\u00e9, \u017ee kdysi d\u00e1vno osoby trp\u00edc\u00ed touto nemoc\u00ed pily zv\u00ed\u0159ec\u00ed krev a vych\u00e1zely pouze v noci. Takov\u00e9 chov\u00e1n\u00ed se mohlo pod\u00edlet na vzniku pov\u011bst\u00ed o up\u00edrech.<\/p>\n

A jak se \u010dlov\u011bk stane up\u00edrem? Hem je plodem procesu, kter\u00fd prob\u00edh\u00e1 p\u0159edev\u0161\u00edm v j\u00e1trech a v kostn\u00ed d\u0159eni. B\u011bhem tohoto procesu se slo\u017eky naz\u00fdvan\u00e9 protoporfyriny p\u0159em\u011b\u0148uj\u00ed v hem. Genetick\u00e9 p\u0159\u00ed\u010diny mohou zp\u016fsobit, \u017ee je proces defektn\u00ed ve v\u00edce kl\u00ed\u010dov\u00fdch bodech a zp\u016fsobuje tak nahromad\u011bn\u00ed protoporfyrin\u016f. V p\u0159\u00edpad\u011b erytropoetick\u00e9 protoporfyrie se v krvi a n\u011bkdy v j\u00e1trech hromad\u00ed protoporfyrin IX.<\/p>\n

Kdy\u017e je protoporfyrin IX vystaven sv\u011btlu, produkuje chemick\u00e9 slo\u017eky, kter\u00e9 po\u0161kozuj\u00ed okoln\u00ed bu\u0148ky. D\u016fsledkem toho jsou otoky, siln\u00e9 bolesti, zarudnut\u00ed k\u016f\u017ee a dokonce vznik puch\u00fd\u0159\u016f pot\u00e9, co se nemocn\u00fd vystavil slunci, i kdyby to bylo jen za okenn\u00edm sklem.<\/p>\n

N\u011bkter\u00e9 genetick\u00e9 p\u0159\u00ed\u010diny ji\u017e byly objeveny, ale n\u011bkter\u00e9 p\u0159\u00edpady erytropoetick\u00e9 protoporfyrie z\u016fst\u00e1vaj\u00ed nevysv\u011btleny. D\u00edky genetick\u00e9 sekvenci \u010dlen\u016f jedn\u00e9 rodiny na severu Francie, kter\u00e1 m\u00e1 erytropoetickou protoporfyrii tohoto typu, objevil t\u00fdm doktora Pawa p\u0159\u00ed\u010dinu choroby: mutaci genu naz\u00fdvan\u00e9ho CLPX.<\/p>\n

V\u011bdci v\u011b\u0159\u00ed, \u017ee objev mutace mo\u017en\u00e1 umo\u017en\u00ed nal\u00e9zt prost\u0159edky, jak tuto chorobu korigovat.<\/p>\n","protected":false},"excerpt":{"rendered":"

Pot\u0159ebuje ke sv\u00e9mu \u017eivotu lidskou krev, vych\u00e1z\u00ed pouze v noci a st\u0159e\u017e\u00ed se sv\u011btla: to jsou n\u011bkter\u00e9 znaky vamp\u00fdra \u010dili up\u00edra. Podle v\u011bdc\u016f v\u0161ak tyto […]<\/a><\/p>\n<\/div>","protected":false},"author":1,"featured_media":29190,"comment_status":"open","ping_status":"closed","sticky":false,"template":"","format":"standard","meta":{"footnotes":""},"categories":[9],"tags":[],"class_list":["post-29188","post","type-post","status-publish","format-standard","has-post-thumbnail","hentry","category-zpravy"],"_links":{"self":[{"href":"https:\/\/www.invarena.cz\/index.php?rest_route=\/wp\/v2\/posts\/29188","targetHints":{"allow":["GET"]}}],"collection":[{"href":"https:\/\/www.invarena.cz\/index.php?rest_route=\/wp\/v2\/posts"}],"about":[{"href":"https:\/\/www.invarena.cz\/index.php?rest_route=\/wp\/v2\/types\/post"}],"author":[{"embeddable":true,"href":"https:\/\/www.invarena.cz\/index.php?rest_route=\/wp\/v2\/users\/1"}],"replies":[{"embeddable":true,"href":"https:\/\/www.invarena.cz\/index.php?rest_route=%2Fwp%2Fv2%2Fcomments&post=29188"}],"version-history":[{"count":0,"href":"https:\/\/www.invarena.cz\/index.php?rest_route=\/wp\/v2\/posts\/29188\/revisions"}],"wp:featuredmedia":[{"embeddable":true,"href":"https:\/\/www.invarena.cz\/index.php?rest_route=\/wp\/v2\/media\/29190"}],"wp:attachment":[{"href":"https:\/\/www.invarena.cz\/index.php?rest_route=%2Fwp%2Fv2%2Fmedia&parent=29188"}],"wp:term":[{"taxonomy":"category","embeddable":true,"href":"https:\/\/www.invarena.cz\/index.php?rest_route=%2Fwp%2Fv2%2Fcategories&post=29188"},{"taxonomy":"post_tag","embeddable":true,"href":"https:\/\/www.invarena.cz\/index.php?rest_route=%2Fwp%2Fv2%2Ftags&post=29188"}],"curies":[{"name":"wp","href":"https:\/\/api.w.org\/{rel}","templated":true}]}}