{"id":3912,"date":"2024-05-23T09:12:57","date_gmt":"2024-05-23T07:12:57","guid":{"rendered":"https:\/\/rijetke-bolesti.com\/?p=3912"},"modified":"2024-05-23T09:12:58","modified_gmt":"2024-05-23T07:12:58","slug":"obiljezavanje-medunarodnog-dana-hereditarnog-angioedema","status":"publish","type":"post","link":"https:\/\/rijetke-bolesti.com\/en\/obiljezavanje-medunarodnog-dana-hereditarnog-angioedema\/","title":{"rendered":"Obilje\u017eavanje me\u0111unarodnog dana hereditarnog angioedema"},"content":{"rendered":"<p>Hereditarni ili nasljedni angioedem (HAE) je rijetki genetski poreme\u0107aj koji je karakteriziran ponavljaju\u0107im epizodama te\u0161kog oticanja (edema) u razli\u010ditim dijelovima tijela, uklju\u010duju\u0107i ruke, noge, lice, crijevnu stijenku i di\u0161ne putove. Ko\u017eni i abdominalni napadaji su naj\u010de\u0161\u0107i oblici HAE. Ovo stanje uzrokovano je nedostatkom ili disfunkcijom C1 inhibitora, proteina koji sudjeluje u regulaciji kompleksnog sustava zgru\u0161avanja krvi i upale. Bez pravilnog funkcioniranja C1 inhibitora, tijelo do\u017eivljava nekontroliranu aktivaciju komplementarnog sustava, \u0161to dovodi do prekomjernog osloba\u0111anja bradikinina, sna\u017enog vazodilatatora koji uzrokuje pro\u0161irenje krvnih \u017eila i curenje, rezultiraju\u0107i edemom.<\/p>\n\n\n\n<p>Glavni simptom HAE-a su ponavljaju\u0107e epizode edema, koje mogu biti nepredvidive i razli\u010dite u te\u017eini. Oticanje lica i di\u0161nih putova mo\u017ee biti opasno po \u017eivot zbog potencijalne opstrukcije di\u0161nih putova. Napadi u trbuhu tako\u0111er su \u010desti i mogu uzrokovati jaku bol, mu\u010dninu, povra\u0107anje i proljev. <strong>HAE se \u010desto pogre\u0161no dijagnosticira zbog svoje rijetkosti i sli\u010dnosti s drugim stanjima, \u0161to dovodi do ka\u0161njenja u pravilnoj dijagnozi i lije\u010denju.<\/strong><\/p>\n\n\n\n<p>Napadaji angioedema ograni\u010davaju bolesnike u obavljanju svakodnevnih aktivnosti, u\u010destalo je izostajanje s posla te iz \u0161kole \u0161to negativno utje\u010de na radnu u\u010dinkovitost, u\u010denje, dru\u0161tveni \u017eivot i obiteljske odnose. Ovi aspekti bolesti zajedno s nepredvidljivosti ataka, izgledom, bolnosti i svijesti o mogu\u0107em lo\u0161em ishodu zna\u010dajno umanjuju kvalitetu \u017eivota ovih bolesnika.<\/p>\n\n\n\n<p>Lije\u010denje HAE-a ima za cilj upravljati simptomima i sprije\u010diti napade. To mo\u017ee uklju\u010divati lijekove po potrebi za lije\u010denje akutnih napada, kao i dugoro\u010dnu profilakti\u010dku terapiju za smanjenje u\u010destalosti i te\u017eine napada, koja je od nedavno dostupna u Hrvatskoj. Osim toga, pacijentima se \u010desto savjetuje da izbjegavaju okida\u010de poput stresa, traume i odre\u0111enih lijekova.<\/p>\n\n\n\n<p>Va\u017eno je da osobe s HAE-om blisko sura\u0111uju sa zdravstvenim stru\u010dnjacima kako bi razvile <strong>personalizirani plan lije\u010denja i imale pristup hitnom lije\u010denju u slu\u010daju te\u0161kih napada<\/strong>.<\/p>\n\n\n<div class=\"wp-block-image\">\n<figure class=\"aligncenter size-large\"><img loading=\"lazy\" decoding=\"async\" width=\"263\" height=\"300\" src=\"https:\/\/rijetke-bolesti.com\/wp-content\/uploads\/2024\/05\/2-3.jpg\" alt=\"\" class=\"wp-image-3917\"\/><\/figure>\n<\/div>\n\n\n<p>Reference:<\/p>\n\n\n\n<p>1. Reshef A, Kidon M, Leibovich I. The story of angioedema: from Quincke to bradykinin. Clin Rev Allergy Immunol 2016;51:121-39.<\/p>\n\n\n\n<p>2. Zuraw BL. Hereditary angioedema. N Engl J Med 2008;359:1027-36.<\/p>\n\n\n\n<p>3. Kaplan AP, Joseph K. Pathogenesis of hereditary angioedema: the role of the bradykinin-forming cascade. Immunol Allergy Clin North Am 2017;37:513-25.<\/p>","protected":false},"excerpt":{"rendered":"<p>Hereditarni ili nasljedni angioedem (HAE) je rijetki genetski poreme\u0107aj koji je karakteriziran ponavljaju\u0107im epizodama te\u0161kog oticanja (edema) u razli\u010ditim dijelovima tijela, uklju\u010duju\u0107i ruke, noge, lice, crijevnu stijenku i di\u0161ne putove. Ko\u017eni i abdominalni napadaji su naj\u010de\u0161\u0107i oblici HAE. Ovo stanje uzrokovano je nedostatkom ili disfunkcijom&#8230;<\/p>","protected":false},"author":3,"featured_media":0,"comment_status":"open","ping_status":"open","sticky":false,"template":"","format":"standard","meta":{"content-type":"","footnotes":""},"categories":[1],"tags":[],"class_list":["post-3912","post","type-post","status-publish","format-standard","hentry","category-uncategorized"],"yoast_head":"<!-- This site is optimized with the Yoast SEO plugin v26.0 - https:\/\/yoast.com\/wordpress\/plugins\/seo\/ -->\n<title>Obilje\u017eavanje me\u0111unarodnog dana hereditarnog angioedema - Hrvatski savez za rijetke bolesti<\/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:\/\/rijetke-bolesti.com\/en\/obiljezavanje-medunarodnog-dana-hereditarnog-angioedema\/\" \/>\n<meta property=\"og:locale\" content=\"en_GB\" \/>\n<meta property=\"og:type\" content=\"article\" \/>\n<meta property=\"og:title\" content=\"Obilje\u017eavanje me\u0111unarodnog dana hereditarnog angioedema - Hrvatski savez za rijetke bolesti\" \/>\n<meta property=\"og:description\" content=\"Hereditarni ili nasljedni angioedem (HAE) je rijetki genetski poreme\u0107aj koji je karakteriziran ponavljaju\u0107im epizodama te\u0161kog oticanja (edema) u razli\u010ditim dijelovima tijela, uklju\u010duju\u0107i ruke, noge, lice, crijevnu stijenku i di\u0161ne putove. 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