{"id":165622,"date":"2022-02-09T08:59:45","date_gmt":"2022-02-09T07:59:45","guid":{"rendered":"https:\/\/sdk.mk\/?p=165622"},"modified":"2022-02-09T08:59:45","modified_gmt":"2022-02-09T07:59:45","slug":"ju-njoftojme-me-semundjet-e-rralla-sindroma-e-hiperimunoglobulinemise-d","status":"publish","type":"post","link":"https:\/\/sdk.mk\/index.php\/maqedonia\/ju-njoftojme-me-semundjet-e-rralla-sindroma-e-hiperimunoglobulinemise-d\/","title":{"rendered":"JU NJOFTOJM\u00cb ME S\u00cbMUNDJET E RRALLA: SINDROMA E HIPERIMUNOGLOBULINEMIS\u00cb D"},"content":{"rendered":"<div class=\"f52428582a4b906b2d2e2a7d733c7d27\" data-index=\"7\" style=\"float: none; margin:10px 0 10px 0; text-align:center;\">\n<!-- MSP Ads Asynchronous JS Tag - Generated with Revive Adserver v5.4.1 -->\r\n<ins data-revive-zoneid=\"167\" data-revive-id=\"060fdb01f6430ec164d3519234aa4967\"><\/ins>\r\n<script async src=\"\/\/panel.ads.com.mk\/www\/delivery\/asyncjs.php\"><\/script>\n<\/div>\n<p class=\"western\" lang=\"mk-MK\"><em><strong>Sindroma e hiperimunoglobulinemis\u00eb D\/Hyperimmunoglobulinemia D syndrome &#8211; HIDS<\/strong><\/em><\/p>\n<p class=\"western\" lang=\"mk-MK\">ICD: E 85.0<\/p>\n<p class=\"western\" lang=\"mk-MK\">Prevalenca: e panjohur<\/p>\n<p class=\"western\" lang=\"mk-MK\">Kjo sindrom\u00eb paraqet nj\u00eb s\u00ebmundje t\u00eb rrall\u00eb autoinflamatore autosomale recesive, e shoq\u00ebruar me episode t\u00eb p\u00ebrs\u00ebritura t\u00eb etheve dhe inflamacionit sistematik.<\/p>\n<p class=\"western\" lang=\"mk-MK\"><strong>Shkaqet:<\/strong><\/p>\n<p class=\"western\" lang=\"mk-MK\">Ndodh si rezultat i varijanteve n\u00eb gjenin MVK q\u00eb kodifikon sintez\u00ebn e enzim\u00ebs mevalonakinaz\u00eb, funksioni i s\u00eb cil\u00ebs \u00ebsht\u00eb t\u00eb shnd\u00ebrroj\u00eb acidin mevalonik n\u00eb mevalonat-5-fosfat. Ky \u00ebsht\u00eb hapi i dyt\u00eb n\u00eb sintez\u00ebn e kolesterolit. Shumica e mutacioneve n\u00eb k\u00ebt\u00eb gjen rezultojn\u00eb n\u00eb prodhimin e nj\u00eb enzime q\u00eb nuk \u00ebsht\u00eb e q\u00ebndrueshme dhe \u00e7on n\u00eb aktivitet t\u00eb reduktuar ose munges\u00eb t\u00eb enzim\u00ebs. Shkalla e aktivitetit t\u00eb enzim\u00ebs s\u00eb mbetur p\u00ebrcakton edhe intensitetin e s\u00ebmundjes. Mekanizmi i sakt\u00eb q\u00eb shkakton reaksione inflamatore dhe shnd\u00ebrrimin e imunoglobulin\u00ebs D n\u00eb serum \u00ebsht\u00eb ende i panjohur. Prevalenca n\u00eb p\u00ebrgjith\u00ebsi \u00ebsht\u00eb e panjohur, por jan\u00eb t\u00eb regjistruar rreth 200 pacient\u00eb n\u00eb t\u00eb gjith\u00eb bot\u00ebn. Shum\u00eb raste jan\u00eb raportuar n\u00eb Holland\u00eb. Nj\u00eb rast i HIDS \u00ebsht\u00eb raportuar edhe n\u00eb Maqedoni.<\/p>\n<p class=\"western\" lang=\"mk-MK\"><strong>Diagnoza:<\/strong><\/p>\n<p class=\"western\" lang=\"mk-MK\">Diagnoza m\u00eb e sakt\u00eb mund t\u00eb konfirmohet me nj\u00eb test gjenetik q\u00eb \u00ebsht\u00eb n\u00eb dispozicion n\u00eb vendin ton\u00eb, n\u00eb Akademin\u00eb e shkencave, ose n\u00eb nj\u00eb laborator privat gjenetik.<\/p>\n<p class=\"western\" lang=\"mk-MK\"><strong>Simptomat:<\/strong><\/p>\n<p class=\"western\" lang=\"mk-MK\">Zakonisht s\u00ebmundja shfaq simptomat e para gjat\u00eb vitit t\u00eb par\u00eb t\u00eb jet\u00ebs. P\u00ebrb\u00ebhet nga episode t\u00eb p\u00ebrs\u00ebritura t\u00eb etheve, dhimbjeve t\u00eb barkut, diarres\u00eb dhe t\u00eb vjellave q\u00eb zakonisht zgjasin 2 deri n\u00eb 7 dit\u00eb dhe p\u00ebrs\u00ebriten \u00e7do 2 deri n\u00eb 8 jav\u00eb.<\/p>\n<p class=\"western\" lang=\"mk-MK\">Gjithashtu shfaqen edhe ndryshime n\u00eb l\u00ebkur\u00eb, nyje limfatike t\u00eb fryra, dhimbje dhe inflamacion n\u00eb ky\u00e7e (artralgji dhe artrit). N\u00eb nj\u00eb episod inflamacioni, numri i rruazave t\u00eb bardha t\u00eb gjakut dhe proteina C-reaktive rriten, k\u00ebshtu q\u00eb shpesh \u00ebsht\u00eb e v\u00ebshtir\u00eb t\u00eb p\u00ebrcaktohet n\u00ebse pasqyra klinike korrespondon me nj\u00eb infeksion t\u00eb v\u00ebrtet\u00eb ose n\u00ebse \u00ebsht\u00eb HIDS. Sapo t\u00eb p\u00ebrfundoj\u00eb episodi, rruazat e bardha t\u00eb gjakut dhe proteina C-reaktive kthehen n\u00eb normale.<\/p>\n<p class=\"western\" lang=\"mk-MK\">Frekuenca e episodeve \u00ebsht\u00eb e theksuar n\u00eb f\u00ebmij\u00ebri dhe zakonisht zvog\u00eblohet me mosh\u00ebn. Sulmet mund t\u00eb ndodhin n\u00eb m\u00ebnyr\u00eb spontane, ose t\u00eb shkaktohen nga vaksinimi, infeksioni, stresi emocional ose fizik. Te k\u00ebta pacient\u00eb zakonisht nuk ka ndryshime n\u00eb rritje dhe zhvillim.<\/p>\n<p class=\"western\" lang=\"mk-MK\"><strong>Nd\u00ebrlikimet:<\/strong><\/p>\n<p class=\"western\" lang=\"mk-MK\">Nd\u00ebrlikimet q\u00eb mund t\u00eb v\u00ebrehen jan\u00eb amiloidoza, adhezionet abdominale, si dhe sindroma e aktivizimit t\u00eb makrofag\u00ebve e cila mund t\u00eb jet\u00eb e rrezikshme p\u00ebr jet\u00ebn.<\/p>\n<p class=\"western\" lang=\"mk-MK\"><strong>Trajtimi:<\/strong><\/p>\n<p class=\"western\" lang=\"mk-MK\">Hapi i par\u00eb n\u00eb zvog\u00eblimin e simptomave t\u00eb etheve dhe dhimbjeve \u00ebsht\u00eb zakonisht p\u00ebrdorimi i barnave anti inflamatore jo steroid (paracetamol, ibuprofen), por ato shpesh nuk funksionojn\u00eb. Hapi i dyt\u00eb jan\u00eb steroidet (pronizone) t\u00eb cilat p\u00ebrdoren p\u00ebr t\u00eb zvog\u00ebluar simptomat ose p\u00ebr t\u00eb ndaluar episodin, por p\u00ebrdorimi i shpesht\u00eb dhe i zgjatur mund t\u00eb shkaktoj\u00eb probleme shtes\u00eb sh\u00ebndet\u00ebsore. Colchicine \u00ebsht\u00eb nj\u00eb ila\u00e7 i sigurt dhe i lir\u00eb q\u00eb p\u00ebrdoret gjithashtu n\u00eb trajtim, por efekti i saj n\u00eb HIDS \u00ebsht\u00eb duksh\u00ebm m\u00eb i dob\u00ebt se ai i s\u00ebmundjeve t\u00eb tjera autoinflamatore.<\/p>\n<p class=\"western\" lang=\"mk-MK\">Trajtimet m\u00eb t\u00eb suksesshme jan\u00eb terapit\u00eb biologjike, t\u00eb tilla si Anakinra dhe Ilaris. Fatkeq\u00ebsisht, k\u00ebto terapi jan\u00eb gjithashtu shum\u00eb t\u00eb shtrenjta dhe k\u00ebrkojn\u00eb p\u00ebrdorim afatgjat\u00eb, duke i b\u00ebr\u00eb ato t\u00eb padisponueshme n\u00eb shum\u00eb vende. Anakinra, nj\u00eb antagonist rekombinant i receptorit IL-1, mund t\u00eb jepet sipas nevoj\u00ebs, gjat\u00eb nj\u00eb episodi, me injeksion subkutan ose si terapi ditore. Ilaris, nj\u00eb beta antitrup monoklonal kund\u00ebr IL-1, gjithashtu injektohet n\u00ebn l\u00ebkur\u00eb, por \u00e7do 4-8 jav\u00eb. K\u00ebto terapi ndihmojn\u00eb n\u00eb kontrollimin e inflamacionit, zvog\u00eblojn\u00eb simptomat e k\u00ebsaj s\u00ebmundjeje ultra t\u00eb rrall\u00eb dhe k\u00ebshtu sigurojn\u00eb nj\u00eb jet\u00eb cil\u00ebsore.<\/p>\n\n<div style=\"font-size: 0px; height: 0px; line-height: 0px; margin: 0; padding: 0; clear: both;\"><\/div>","protected":false},"excerpt":{"rendered":"<p>Sindroma e hiperimunoglobulinemis\u00eb D\/Hyperimmunoglobulinemia D syndrome &#8211; HIDS ICD: E 85.0 Prevalenca: e panjohur Kjo sindrom\u00eb paraqet nj\u00eb s\u00ebmundje t\u00eb [&hellip;]<\/p>\n","protected":false},"author":22,"featured_media":165623,"comment_status":"closed","ping_status":"closed","sticky":false,"template":"","format":"standard","meta":{"_mo_disable_npp":"no","inline_featured_image":false},"categories":[3087],"tags":[56950],"imageURL":"https:\/\/sdk.mk\/wp-content\/uploads\/2022\/02\/09-Hyperimmunoglobulinemia-D-syndrome-650x366.png","thumbURL":"https:\/\/sdk.mk\/wp-content\/uploads\/2022\/02\/09-Hyperimmunoglobulinemia-D-syndrome-250x141.png","categoryNames":["Maqedonia"],"feature_caption":"","subtitle":"","views":"210","source":"\u0421\u043a\u043e\u043f\u0458\u0435, (\u0421\u0410\u041a\u0410\u041c\u0414\u0410\u041a\u0410\u0416\u0410\u041c.\u041c\u041a)","yoast_head":"<!-- This site is optimized with the Yoast SEO Premium plugin v15.0 - https:\/\/yoast.com\/wordpress\/plugins\/seo\/ -->\n<title>JU NJOFTOJM\u00cb ME S\u00cbMUNDJET E RRALLA: SINDROMA E HIPERIMUNOGLOBULINEMIS\u00cb D - \u0421\u0430\u043a\u0430\u043c \u0414\u0430 \u041a\u0430\u0436\u0430\u043c<\/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:\/\/sdk.mk\/index.php\/maqedonia\/ju-njoftojme-me-semundjet-e-rralla-sindroma-e-hiperimunoglobulinemise-d\/\" \/>\n<meta property=\"og:locale\" content=\"mk_MK\" \/>\n<meta property=\"og:type\" content=\"article\" \/>\n<meta property=\"og:title\" content=\"JU NJOFTOJM\u00cb ME S\u00cbMUNDJET E RRALLA: SINDROMA E HIPERIMUNOGLOBULINEMIS\u00cb D - \u0421\u0430\u043a\u0430\u043c \u0414\u0430 \u041a\u0430\u0436\u0430\u043c\" \/>\n<meta property=\"og:description\" content=\"Sindroma e hiperimunoglobulinemis\u00eb D\/Hyperimmunoglobulinemia D syndrome &#8211; 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