{"id":106674,"date":"2020-02-11T08:08:43","date_gmt":"2020-02-11T07:08:43","guid":{"rendered":"https:\/\/sdk.mk\/?p=106674"},"modified":"2020-02-16T08:46:36","modified_gmt":"2020-02-16T07:46:36","slug":"ju-njoftojme-me-semundjet-e-rralla-porfiria","status":"publish","type":"post","link":"https:\/\/sdk.mk\/index.php\/uncategorized-sq\/ju-njoftojme-me-semundjet-e-rralla-porfiria\/","title":{"rendered":"JU NJOFTOJM\u00cb ME S\u00cbMUNDJET E RRALLA: PORFIRIA"},"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><a href=\"https:\/\/www.youtube.com\/watch?v=Qo4IBSc0xC8&amp;list=PLPGcigDEy-qj0I1um2ByEqy4a1IdVRjzf&amp;index=12&amp;t=0s\" target=\"_blank\" rel=\"noopener noreferrer\"><em><strong>Porfiria<\/strong><\/em><\/a><br \/>\nPorphyria<\/p>\n<p>KNS \u0415 80.0, \u0415 80.1, \u0415 80.2<br \/>\nPorfiria paraqet nj\u00eb grup nga m\u00eb s\u00eb paku tet\u00eb s\u00ebmundje t\u00eb ndryshme (t\u00eb trash\u00ebguara apo t\u00eb fituara), karakteristikat e s\u00eb cilave dallojn\u00eb shum\u00eb. Karakteristik\u00eb e p\u00ebrbashk\u00ebt e t\u00eb gjitha porfirive \u00ebsht\u00eb grumbullimi i \u201cporfirinave\u201d ose prekursor\u00ebve t\u00eb tyre n\u00eb organiz\u00ebm. Edhe pse porfirinat normalisht gjenden n\u00eb organiz\u00ebm, por nuk grumbullohen n\u00eb organiz\u00ebm t\u00eb sh\u00ebndetsh\u00ebm.<br \/>\nZhvillimi i porfiris\u00eb \u00ebsht\u00eb i shkaktuar nga \u00e7rregullimi, n\u00eb fakt mungesa e enzim\u00ebs (protein\u00eb q\u00eb i stimulon reaksionet n\u00eb organiz\u00ebm) e cila merr pjes\u00eb n\u00eb biosintez\u00ebn e hemit, dhe rezulton me grumbullim e molekulave t\u00eb porfirin\u00ebs n\u00eb organiz\u00ebm. Emri rrjedh nga fjala greke \u201cporfirus\u201d q\u00eb do t\u00eb thot\u00eb ngjyr\u00eb vjollc\u00eb (ngjyr\u00eb e hapur e kuqe \u2013 vjollc\u00eb).<br \/>\nPorfiria \u00ebsht\u00eb s\u00ebmundje e rrall\u00eb shkaku kryesor i s\u00eb cil\u00ebs jan\u00eb mutacionet gjenetike.<br \/>\nSipas t\u00eb dh\u00ebnave statistikore, incidenca \u00ebsht\u00eb 2 raste n\u00eb 100.000 banor\u00eb. N\u00eb vendet e Europ\u00ebs veriore si Anglia, Irlanda dhe Suedia, porfiria shfaqet m\u00eb shpesh \u2013 2 t\u00eb s\u00ebmur\u00eb n\u00eb 10.000 banor\u00eb. Porfiria gjithashtu njihet si s\u00ebmundja vampire nga viti 1985 kur biokimisti Dejfin Dolfin e v\u00ebrtetoi q\u00eb rr\u00ebfimet p\u00ebr vampir\u00ebt jan\u00eb n\u00eb t\u00eb v\u00ebrtet\u00eb rr\u00ebfime p\u00ebr njer\u00ebz q\u00eb vuajn\u00eb nga porfiria. Argumentet e tij jan\u00eb k\u00ebta: pacient\u00ebt jan\u00eb jasht\u00ebzakonisht t\u00eb ndjesh\u00ebm ndaj drit\u00ebs pasi q\u00eb ajo mund shkaktoj\u00eb njolla (madje edhe nj\u00eb eksponim i vog\u00ebl \u2013 shpesh dalin jasht\u00eb vet\u00ebm nat\u00ebn), l\u00ebkura mund t\u00eb jet\u00eb e mbushur me shenja plag\u00ebsh, dh\u00ebmb\u00ebt si t\u00eb qenit mund t\u00eb jen\u00eb shum\u00eb t\u00eb shprehura (nofulla dhe dh\u00ebmb\u00ebt mund t\u00eb jen\u00eb shum\u00eb t\u00eb tendosura).<br \/>\nPorfiria &#8211; shkaqet<br \/>\nPorfiria m\u00eb shpesh shfaqet si pasoj\u00eb e munges\u00ebs s\u00eb cil\u00ebsdo nga shtat\u00eb enzimat n\u00eb rrug\u00ebn e sintez\u00ebs s\u00eb hemit. \u00c7donj\u00ebra nga shtat\u00eb enzimat kodohet nga nj\u00eb gjen specifik. Mutacionet eventuale e nd\u00ebrpresin aktivitetin e nj\u00ebr\u00ebs nga enzimet. Shumica e porfirive jan\u00eb trash\u00ebguese. \u00cbsht\u00eb e mundshme t\u00eb trash\u00ebgohet gjeni abnormal nga nj\u00ebri apo nga t\u00eb dy prind\u00ebrit. Por kjo nuk do t\u00eb thot\u00eb q\u00eb do zhvillohet porfiri, por b\u00ebhet fjal\u00eb p\u00ebr nj\u00eb person q\u00eb e bart gjenin me mutacion. Kjo gj\u00ebndje njihet si porfiri latente.<br \/>\nSimptomat e porfiris\u00eb<br \/>\nEkzistojn\u00eb dy lloje kryesore t\u00eb porfirive \u2013 akute dhe t\u00eb l\u00ebkur\u00ebs. Porfiria akute kryesisht ndikon n\u00eb sistemin nervor, kurse porfiria e l\u00ebkur\u00ebs, si\u00e7 sygjeron edhe vet\u00eb emri, shkakton d\u00ebmtime t\u00eb l\u00ebkur\u00ebs. Disa lloje t\u00eb porfiris\u00eb mund t\u00eb jen\u00eb kombinime nga k\u00ebto dy lloje dhe mund t\u00eb prekin edhe sistemin nervor edhe l\u00ebkur\u00ebn.<br \/>\nSimptomat e porfiris\u00eb akute<br \/>\nPorfiria akute \u00ebsht\u00eb karakteristike p\u00ebr grat\u00eb n\u00eb periudh\u00ebn nga menstruacioni i par\u00eb gjer te menopauza.<br \/>\nSimptomat kryesore t\u00eb porfiris\u00eb akute jan\u00eb:<br \/>\n&#8211; dhimbje e fort\u00eb barku,<br \/>\n&#8211; fryerje,<br \/>\n&#8211; dhimbje n\u00eb gjoks, n\u00eb k\u00ebmb\u00eb dhe shpin\u00eb,<br \/>\n&#8211; kapsll\u00ebk,<br \/>\n&#8211; nauze dhe vjellje,<br \/>\n&#8211; tahikardi (rrahje e shpejt\u00eb e zemr\u00ebs),<br \/>\n&#8211; tension t\u00eb lart\u00eb t\u00eb gjakut,<br \/>\n&#8211; shqet\u00ebsim,<br \/>\n&#8211; t\u00eb mundshme, por t\u00eb rralla, ndryshime n\u00eb sjellje (dezorientim dhe paranoj\u00eb),<br \/>\n&#8211; \u00e7rregullime respiratore,<br \/>\n&#8211; urin\u00eb t\u00eb kuqe ose ngjyr\u00eb kafe.<br \/>\nSimptomat e porfiris\u00eb kronike:<br \/>\nPorfiria e l\u00ebkur\u00ebs kryesisht i referohet s\u00ebmundjeve simptomat e t\u00eb cilave jan\u00eb rezultat i mbindjeshm\u00ebris\u00eb ndaj drit\u00ebs s\u00eb diellit. Sulmet te ky lloj i porfiris\u00eb mund t\u00eb zgjasin disa dit\u00eb, kurse simptomat e k\u00ebsaj s\u00ebmundje shfaqen qysh nga f\u00ebmij\u00ebria.<br \/>\nSimptomat e porfiris\u00eb s\u00eb l\u00ebkur\u00ebs:<br \/>\n&#8211; ndjeshm\u00ebri e l\u00ebkur\u00ebs ndaj drit\u00ebs s\u00eb diellit,<br \/>\n&#8211; skuqje e dhimbshme e l\u00ebkur\u00ebs (eritem) dhe \u00ebnjt\u0458\u0435 (edem),<br \/>\n&#8211; flluska,<br \/>\n&#8211; kruarje,<br \/>\n&#8211; l\u00ebkur\u00eb e ndjeshme dhe fragile,<br \/>\n&#8211; shenja<br \/>\n&#8211; higroskopi e lart\u00eb (aft\u00ebsi p\u00ebr ta mbajtur ujin)<br \/>\n&#8211; urin\u00eb t\u00eb kuqe ose ngjyr\u00eb kafe.<br \/>\nPorfiria \u2013 diagnoza:<br \/>\nDiagnoza vehet n\u00eb baz\u00eb t\u00eb p\u00ebrd\u00ebftimit t\u00eb porfirin\u00ebs n\u00eb urin\u00eb dhe gjak. Kjo analiz\u00eb mund ta caktoj\u00eb edhe llojin e porfiris\u00eb. Disa pacient\u00eb kan\u00eb vlera normale ose pak t\u00eb rritura t\u00eb porfirin\u00ebs, ashtu q\u00eb mostrat duhet t\u00eb merren gjat\u00eb ose shkurt koh\u00eb pas kriz\u00ebs.<br \/>\nAnaliza gjenetike \u00ebsht\u00eb e sigurt dhe m\u00eb s\u00eb shumti e favorizon trajtimin diagnostik t\u00eb familjeve me mutacione t\u00eb njohura t\u00eb gjeneve. Gjithashtu \u00ebsht\u00eb i mundsh\u00ebm testimi prenatal p\u00ebr porfiri, por praktikohet shum\u00eb rrall\u00eb.<br \/>\nPrognoza dhe trajtimi i porfiris\u00eb:<br \/>\nTretmani i porfiris\u00eb do t\u00eb varet nga forma e s\u00ebmundjes. Rastet m\u00eb t\u00eb v\u00ebshtira t\u00eb porfiris\u00eb kan\u00eb nevoj\u00eb p\u00ebr hospitalizim. Prognoza e pacient\u00ebve me simptome t\u00eb porfiris\u00eb \u00ebsht\u00eb duksh\u00ebm m\u00eb e favorshme me p\u00ebrmir\u00ebsimin e terapis\u00eb dhe me vet\u00ebkontrollin. Sidoqoft\u00eb, num\u00ebr i madh i njer\u00ebzve q\u00eb vuajn\u00eb nga porfiria vazhdojn\u00eb t\u00eb ken\u00eb kriza, q\u00eb shpie gjer te d\u00ebmtimi progresiv me pasoja t\u00eb p\u00ebrhershme si\u00e7 \u00ebsht\u00eb insuficienca e veshkave.<br \/>\nNevoja p\u00ebr analgjetik\u00eb te porfiria mund t\u00eb kaloj\u00eb n\u00eb varshm\u00ebri. Glukoza, q\u00eb futet n\u00eb trupin e pacientit p\u00ebrmes kateterit venoz central, ndihmon n\u00eb leht\u00ebsimin dhe modifikim e simptomave.<br \/>\nTrajtimi i porfiris\u00eb akute p\u00ebrfshin:<br \/>\n&#8211; p\u00ebrdorimi i analgjetik\u00ebve dhe barnave kund\u00ebr nauzes\u00eb dhe vjelljes (antiemetik\u00eb),<br \/>\n&#8211; trajtim i infeksioneve dhe s\u00ebmundjeve tjera q\u00eb mund t\u00eb shkaktojn\u00eb simptoma,<br \/>\n&#8211; administrim intravenoz t\u00eb glukoz\u00ebs<br \/>\n&#8211; administrim t\u00eb hemin\u00ebs (Fe).<br \/>\nTretmani i porfiris\u00eb s\u00eb l\u00ebkur\u00ebs n\u00ebnkupton shmangjen e diellit (UV drit\u00ebs) dhe tretmanet q\u00eb vijojn\u00eb:<br \/>\n&#8211; flebotomi (l\u00ebshim i gjakut nga venat),<br \/>\n&#8211; p\u00ebrdorim t\u00eb antimalarik\u00ebve<br \/>\n&#8211; beta-karoten,<br \/>\n&#8211; suplemente t\u00eb vitamin\u00ebs D.<br \/>\nT\u00eb prekurit nga porfiria shum\u00eb shpesh k\u00ebrkojn\u00eb ndihm\u00eb psikologjike. Ndryshimet e trupit jan\u00eb burim i vazhduesh\u00ebm i zhg\u00ebnjimit, depresionit, trishtimit, frik\u00ebs dhe pasiguris\u00eb n\u00eb lidhje me s\u00ebmundjen. Pacient\u00ebt me format progresive t\u00eb porfiris\u00eb shum\u00eb v\u00ebshtir\u00eb e pranojn\u00eb s\u00ebmundjen dhe, dita dit\u00ebs p\u00ebrpiqen t\u00eb m\u00ebsojn\u00eb t\u00eb jetojn\u00eb me \u201cs\u00ebmundjen e re\u201d.<br \/>\nPorfiria &#8211; dieta<br \/>\nNjer\u00ebzit q\u00eb kan\u00eb predispozit\u00eb gjenetike p\u00ebr zhvillim t\u00eb porfiris\u00eb, \u00ebsht\u00eb e sigurt q\u00eb duhet ta menjanojn\u00eb ushqimin q\u00eb p\u00ebrmban:<br \/>\n&#8211; glutamat t\u00eb mononatriumit,<br \/>\n&#8211; p\u00ebrb\u00ebrje sulfurike (qep\u00eb, hudh\u00ebr, brokoli, lak\u00ebr dhe perime tjera),<br \/>\n&#8211; salicilate (shum\u00eb lloje t\u00eb frutav\u0435),<br \/>\n&#8211; oksalate (shum\u00eb lloje t\u00eb frutave, zarzavateve dhe ushqimi bimor),<br \/>\n&#8211; acidi sialik,<br \/>\n&#8211; ushqim q\u00eb \u00e7liron histamin\u00eb,<br \/>\n&#8211; gluten,<br \/>\n&#8211; lecitin (i fituar nga veza dhe soja),<br \/>\n&#8211; perime (domate, patate, speca),<br \/>\n&#8211; yndyr\u00ebra dhe\/ose vaj\u00ebra.<br \/>\nN\u00eb rast se keni porfiri, duhet t\u00eb konsultoheni me mjekun tuaj q\u00eb tu paralajm\u00ebroj p\u00ebr ushqimin dhe shprehit\u00eb gjat\u00eb jet\u00ebs t\u00eb cilat jan\u00eb t\u00eb d\u00ebmshme p\u00ebr ju, kurse me ndihm\u00ebn e nutricionistit, duhet t\u00eb fitoni plan adekuat p\u00ebr ushqimin tuaj.<\/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>Porfiria Porphyria KNS \u0415 80.0, \u0415 80.1, \u0415 80.2 Porfiria paraqet nj\u00eb grup nga m\u00eb s\u00eb paku tet\u00eb s\u00ebmundje t\u00eb [&hellip;]<\/p>\n","protected":false},"author":24,"featured_media":106672,"comment_status":"closed","ping_status":"closed","sticky":false,"template":"","format":"standard","meta":{"_mo_disable_npp":"","inline_featured_image":false},"categories":[1794],"tags":[],"imageURL":"https:\/\/sdk.mk\/wp-content\/uploads\/2020\/02\/11-Porphyria-650x366.png","thumbURL":"https:\/\/sdk.mk\/wp-content\/uploads\/2020\/02\/11-Porphyria-250x141.png","categoryNames":["Uncategorized"],"feature_caption":"","subtitle":"","views":"3437","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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