{"id":165227,"date":"2022-02-03T09:46:06","date_gmt":"2022-02-03T08:46:06","guid":{"rendered":"https:\/\/sdk.mk\/?p=165227"},"modified":"2022-02-03T09:46:06","modified_gmt":"2022-02-03T08:46:06","slug":"ju-njoftojme-me-semundjet-e-rralla-sindroma-kongjenitale-e-qt-se-se-gjate","status":"publish","type":"post","link":"https:\/\/sdk.mk\/index.php\/maqedonia\/ju-njoftojme-me-semundjet-e-rralla-sindroma-kongjenitale-e-qt-se-se-gjate\/","title":{"rendered":"JU NJOFTOJM\u00cb ME S\u00cbMUNDJET E RRALLA: SINDROMA KONGJENITALE E QT-S\u00cb S\u00cb GJAT\u00cb"},"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 kongjenitale e QT-s\u00eb s\u00eb gjat\u00eb\/Congenital long QT syndrome (LQTS)<\/strong><\/em><\/p>\n<p class=\"western\" lang=\"mk-MK\">ICD: I 45.8<\/p>\n<p class=\"western\" lang=\"mk-MK\">Prevalenca: e panjohur<\/p>\n<p class=\"western\" lang=\"mk-MK\">Prevalenca e form\u00ebs klasike: 1\/5.000<\/p>\n<p class=\"western\" lang=\"mk-MK\">Sindroma kongjenitale e QT-s\u00eb s\u00eb gjat\u00eb p\u00ebrfshin nj\u00eb grup t\u00eb \u00e7rregullimeve t\u00eb rralla, gjenetike, t\u00eb trash\u00ebguara dhe \u00ebsht\u00eb nj\u00eb nga shkaqet m\u00eb t\u00eb zakonshme t\u00eb vdekjes s\u00eb papritur tek t\u00eb rinjt\u00eb. Kjo ndodh p\u00ebr shkak t\u00eb nj\u00eb mutacioni n\u00eb gjenet q\u00eb kodifikojn\u00eb disa kanale jonesh t\u00eb ndryshme n\u00eb membran\u00ebn qelizore q\u00eb rregullojn\u00eb repolarizimin e qelizave t\u00eb miokardit. Prania e k\u00ebsaj sindrome mund t\u00eb \u00e7oj\u00eb n\u00eb nj\u00eb lloj takikardie ventrikulare t\u00eb papritur, k\u00ebrc\u00ebnuese p\u00ebr jet\u00ebn. Rrahjet e zemr\u00ebs jan\u00eb jasht\u00ebzakonisht t\u00eb shpejta, pamja e komplekseve n\u00eb EKG po ndryshon vazhdimisht duke i ngjar\u00eb nj\u00eb val\u00eb sinusore dhe duke e b\u00ebr\u00eb t\u00eb pamundur pompimin efektiv t\u00eb gjakut nga zemra.<\/p>\n<p class=\"western\" lang=\"mk-MK\"><strong>Shkaqet:<\/strong><\/p>\n<p class=\"western\" lang=\"mk-MK\">Jan\u00eb identifikuar disa gjene q\u00eb ndikojn\u00eb n\u00eb intervalin QT, k\u00ebshtu q\u00eb ekzistojn\u00eb disa variante t\u00eb k\u00ebsaj sindrome. Format familjare t\u00eb sindrom\u00ebs s\u00eb QT-s\u00eb s\u00eb gjat\u00eb, t\u00eb cilat shoq\u00ebrohen me nj\u00eb incidenc\u00eb t\u00eb lart\u00eb t\u00eb aritmive t\u00eb rrezikshme, vdekje t\u00eb papritur dhe q\u00eb shpesh ndodhin te disa an\u00ebtar\u00eb t\u00eb nj\u00eb familjeje, p\u00ebrfshijn\u00eb sindrom\u00ebn \u0408erwell dhe Lange-Nielsen, e cila trash\u00ebgohet n\u00eb nj\u00eb m\u00ebnyr\u00eb autosomale recesive dhe n\u00eb shumic\u00ebn e rasteve shoq\u00ebrohen me humbje t\u00eb d\u00ebgjimit. Sindroma Romano-Ward \u00ebsht\u00eb m\u00eb e shpesht\u00eb, trash\u00ebgohet n\u00eb m\u00ebnyr\u00ebn autosomale dominante, dhe \u00ebsht\u00eb gjithashtu nj\u00eb form\u00eb familjare.<\/p>\n<p class=\"western\" lang=\"mk-MK\"><strong>Simptomat:<\/strong><\/p>\n<p class=\"western\" lang=\"mk-MK\">Personat me k\u00ebt\u00eb sindrom\u00eb n\u00eb elektrokardiogram\u00eb kan\u00eb nj\u00eb interval QT t\u00eb zgjatur. Simptomat shfaqen vet\u00ebm kur pacienti zhvillon nj\u00eb episod t\u00eb takikardis\u00eb ventrikulare dhe varen nga koh\u00ebzgjatja e aritmis\u00eb. Marramendje e shkurt\u00eb mund t\u00eb jet\u00eb simptoma e vetme. N\u00ebse zgjat m\u00eb shum\u00eb se 10 sekonda, ndodh sinkopa, humbje afatshkurt\u00ebr e vet\u00ebdijes, deri n\u00eb vdekjen e papritur kardiake t\u00eb pacientit brenda pak minutash, n\u00ebse nuk i jepet kujdes mjek\u00ebsor urgjent. Kjo \u00ebsht\u00eb simptoma e par\u00eb te 1 n\u00eb 10 pacient\u00eb me sindrom\u00eb te QT-s\u00eb s\u00eb gjat\u00eb. Simptomat mund t\u00eb fillojn\u00eb gjat\u00eb vitit t\u00eb par\u00eb t\u00eb jet\u00ebs dhe t\u00eb b\u00ebhen m\u00eb t\u00eb shpeshta gjat\u00eb mosh\u00ebs shkollore. Djemt\u00eb zakonisht do t\u00eb p\u00ebrjetojn\u00eb episodin e par\u00eb t\u00eb sinkop\u00ebs n\u00eb mosh\u00ebn mesatare 11 vje\u00e7, dhe vajzat n\u00eb mosh\u00ebn 16 vje\u00e7. Episodet e zemr\u00ebs mund t\u00eb shkaktohen nga stresi fizik dhe emocional (ushtrime, zem\u00ebrim, frik\u00eb, g\u00ebzim, eksitim, zhytje, etj.). Disa nga k\u00ebto episode kan\u00eb ndodhur n\u00eb raste t\u00eb ve\u00e7anta edhe gjat\u00eb gjumit ose pushimit.<\/p>\n<p class=\"western\" lang=\"mk-MK\"><strong>Trajtimi:<\/strong><\/p>\n<p class=\"western\" lang=\"mk-MK\">Personat me sindrom\u00ebn e QT-s\u00eb s\u00eb gjat\u00eb k\u00ebshillohen ti kufizojn\u00eb aktivitetet e tyre n\u00eb sporte me intensitet t\u00eb ul\u00ebt n\u00ebse kan\u00eb nj\u00eb histori t\u00eb humbjes s\u00eb vet\u00ebdijes. sinkop\u00eb, ose reanimim nga sulmi n\u00eb zem\u00ebr, si dhe n\u00eb rastet kur intervali QT \u00ebsht\u00eb shum\u00eb i zgjatur (470 milisekonda te burrat, 480 milisekonda te grat\u00eb). Shum\u00eb pacient\u00eb me k\u00ebt\u00eb sindrom\u00eb trajtohen me beta-bllokues. Disa medikamente antiaritmike, ila\u00e7et kund\u00ebr depresionit, antibiotik\u00ebt, si eritromicina, klaritromicina, dhe azitromicina, shpesh e zgjasin intervalin QT. Prandaj, k\u00ebto barna q\u00eb shkaktojn\u00eb nj\u00eb interval QT t\u00eb zgjatur duhet t\u00eb shmangen.<\/p>\n<p class=\"western\" lang=\"mk-MK\">P\u00ebr shumic\u00ebn e pacient\u00ebve, trajtimi m\u00eb i mir\u00eb \u00ebsht\u00eb nj\u00eb defibrilator i implantuar. Kjo pajisje duhet t\u00eb p\u00ebrdoret te pacient\u00ebt q\u00eb kan\u00eb mbijetuar nj\u00eb sulm n\u00eb zem\u00ebr dhe te pacient\u00ebt q\u00eb kan\u00eb pasur sinkop\u00eb p\u00ebr shkak t\u00eb vet\u00eb sindrom\u00ebs s\u00eb QT-s\u00eb s\u00eb gjat\u00eb, ve\u00e7an\u00ebrisht n\u00ebse sinkopa shfaqet p\u00ebrkund\u00ebr asaj q\u00eb ata po trajtohen me beta-bllokues.<\/p>\n<p class=\"western\" lang=\"mk-MK\">P\u00ebr ata q\u00eb nuk mund t\u00eb tolerojn\u00eb beta-bllokuesit ose q\u00eb vazhdojn\u00eb t\u00eb ken\u00eb sulme n\u00eb zem\u00ebr edhe pse pranojn\u00eb terapi, mund t\u00eb kryhet denervimi kardio-simpatik i majt\u00eb. Diagnoza prenatale vjen n\u00eb konsiderim kur nj\u00ebri prind vuan nga sindroma e QT-s\u00eb s\u00eb gjat\u00eb dhe k\u00ebtyre familjeve t\u00eb prekura duhet t&#8217;u ofrohet k\u00ebshillim gjenetik.<\/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 kongjenitale e QT-s\u00eb s\u00eb gjat\u00eb\/Congenital long QT syndrome (LQTS) ICD: I 45.8 Prevalenca: e panjohur Prevalenca e form\u00ebs klasike: [&hellip;]<\/p>\n","protected":false},"author":22,"featured_media":165228,"comment_status":"closed","ping_status":"closed","sticky":false,"template":"","format":"standard","meta":{"_mo_disable_npp":"no","inline_featured_image":false},"categories":[3087],"tags":[56828],"imageURL":"https:\/\/sdk.mk\/wp-content\/uploads\/2022\/02\/03-Congenital-long-QT-syndrome-LQTS-1-650x366.png","thumbURL":"https:\/\/sdk.mk\/wp-content\/uploads\/2022\/02\/03-Congenital-long-QT-syndrome-LQTS-1-250x141.png","categoryNames":["Maqedonia"],"feature_caption":"","subtitle":"","views":"218","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 - 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