{"id":200393,"date":"2024-04-18T05:46:50","date_gmt":"2024-04-18T01:46:50","guid":{"rendered":""},"modified":"-0001-11-30T00:00:00","modified_gmt":"-0001-11-29T20:00:00","slug":"","status":"publish","type":"post","link":"https:\/\/ucnoqta.az\/?p=200393","title":{"rendered":"Gen terapiyas\u0131 hemofiliyada \u00fcmid verir"},"content":{"rendered":"

17 aprel Ümumdünya Hemofiliya Günüdür. Hemofiliya, laxtalanma faktorlar\u0131n\u0131n çat\u0131\u015fmazl\u0131\u011f\u0131 s\u0259b\u0259bind\u0259n qan\u0131n laxtalana bilm\u0259diyi nadir bir x\u0259st\u0259likdir. Da\u015f\u0131y\u0131c\u0131 qad\u0131nlar\u0131n geni u\u015faqlar\u0131na ötürm\u0259 \u015fans\u0131 50 faizdir. <\/strong><\/p>\n

Ucnoqta.az x\u0259b\u0259r verir ki, hematologiya müt\u0259x\u0259ssisi prof. dr. Mustafa Köro\u011flu, hemofiliyan\u0131n müalic\u0259sind\u0259 ya\u015fanan ç\u0259tinlikl\u0259r\u0259 gör\u0259 ehtiyat t\u0259dbirl\u0259rinin al\u0131nmas\u0131n\u0131n prioritet oldu\u011funu vur\u011fulayaraq, hemofiliya il\u0259 ba\u011fl\u0131 b\u0259zi m\u0259lumatlar\u0131 verdi:<\/p>\n

 "Qan\u0131m\u0131zda f\u0259rqli funksiyalar\u0131 olan zülallar var. Onlar\u0131n ham\u0131s\u0131n\u0131n f\u0259rqli funksiyalar\u0131 var. B\u0259zi zülallar h\u0259ddind\u0259n art\u0131q x\u0259st\u0259liyin qar\u015f\u0131s\u0131n\u0131 almaq xüsusiyy\u0259tin\u0259 malikdir. Travma ya\u015fad\u0131\u011f\u0131m\u0131z zaman damarlar\u0131m\u0131zdan qan tökülm\u0259si bu zülallar\u0131n laxtalanmas\u0131ndan m\u0259suldur. Bu amill\u0259r\u0259 faktorlar deyilir. Normal h\u0259yatda bu faktorlar i\u015fl\u0259mir, y\u0259ni h\u0259r\u0259k\u0259tsizdirl\u0259r. \u018fg\u0259r i\u015fl\u0259s\u0259ydil\u0259r, qan\u0131m\u0131z donar, damarlar v\u0259 qan dövran\u0131 davam etm\u0259y\u0259c\u0259k, bir yandan da qan dövran\u0131 dursa da, b\u0259d\u0259nin dig\u0259r yerl\u0259rind\u0259 qanaxma ba\u015flard\u0131. Ancaq b\u0259zi insanlarda bu amill\u0259rd\u0259 çat\u0131\u015fmazl\u0131qlar, h\u0259ddind\u0259n art\u0131ql\u0131qlar v\u0259 ya anormall\u0131qlar ola bil\u0259r".<\/p>\n

 Koaqulyasiya faktoru x\u0259st\u0259likl\u0259rinin \u0259ks\u0259riyy\u0259tinin irsi x\u0259st\u0259likl\u0259r qrupunda oldu\u011funu söyl\u0259y\u0259n prof. Dr. Köro\u011flu \u0259ks\u0259r hallarda x\u0259st\u0259lik ail\u0259vi xüsusiyy\u0259tl\u0259r\u0259 malikdir v\u0259 qüsurlu geni da\u015f\u0131yan valideynd\u0259n miras yolu il\u0259 g\u0259l\u0259c\u0259k n\u0259sl\u0259 ötürüldüyünü deyir.<\/p>\n

Professor bildirib ki, hemofiliyal\u0131 x\u0259st\u0259l\u0259rin qan\u0131nda kifay\u0259t q\u0259d\u0259r laxtalanma faktoru olmad\u0131\u011f\u0131ndan, yaralanan zaman qanlar\u0131 normal laxtalanm\u0131r. Dr. Köro\u011flu deyib: "K\u0259silmi\u015f v\u0259 z\u0259d\u0259l\u0259nmi\u015f nahiy\u0259d\u0259 qanaxma daha uzun sürür. Travmadan sonra gec qanaxma ba\u015f ver\u0259 bil\u0259r. Kiçik z\u0259d\u0259l\u0259r ümumiyy\u0259tl\u0259 ciddi problem yaratm\u0131r. Orqanlar\u0131n, \u0259z\u0259l\u0259l\u0259rin v\u0259 ya oynaqlar\u0131n görünm\u0259y\u0259n daxili hiss\u0259l\u0259rind\u0259 meydana g\u0259l\u0259n qanaxma ciddi narahatl\u0131q do\u011furur. Planl\u0131 v\u0259 ani inki\u015faflar "Bel\u0259 hallarda z\u0259ruri c\u0259rrahi \u0259m\u0259liyyatlar zaman\u0131 v\u0259 ya sonras\u0131nda qanaxma a\u011f\u0131rla\u015fmalar\u0131n\u0131n inki\u015faf riski d\u0259 çox yüks\u0259kdir." Daxili qanaxman\u0131n orqan v\u0259 toxumalara z\u0259r\u0259r ver\u0259 bil\u0259c\u0259yi üçün h\u0259yati t\u0259hlük\u0259si oldu\u011funu ifad\u0259 ed\u0259n Prof. Dr. Köro\u011flu, "Qan\u0131n laxtalanma prosesini yava\u015flatan hemofiliya x\u0259st\u0259liyi ciddi f\u0259sadlara yol aça bil\u0259r. Oynaqda qanaxma xroniki hala g\u0259ldiyi üçün f\u0259rdl\u0259r qollar\u0131n\u0131 v\u0259 ayaqlar\u0131n\u0131 düzgün h\u0259r\u0259k\u0259t etdir\u0259 bilm\u0259y\u0259 ba\u015flay\u0131rlar. Oynaq deformasiyalar\u0131 inki\u015faf etdiyi üçün x\u0259st\u0259 h\u0259mi\u015f\u0259lik \u0259lil qala bil\u0259r”.<\/p>\n

DA\u015eIYICI QADINLAR BUNU U\u015eAQLARINA KEÇ\u0130R\u0130R
\nHemofiliyan\u0131n ümumiyy\u0259tl\u0259 genetik bir x\u0259st\u0259lik oldu\u011funu vur\u011fulayan Prof. Dr. Köro\u011flu deyib: "Ancaq x\u0259st\u0259liyin müxt\u0259lif növl\u0259ri v\u0259 \u015fidd\u0259t d\u0259r\u0259c\u0259l\u0259ri var. Qanaman\u0131n gedi\u015fi buna gör\u0259 d\u0259yi\u015fir. B\u0259zi hallar do\u011fu\u015fdan a\u011f\u0131r keçir. Dig\u0259rl\u0259ri normal h\u0259yatlar\u0131n\u0131 davam etdirirl\u0259r v\u0259 qanaxma yaln\u0131z h\u0259yat\u0131n mü\u0259yy\u0259n dövrl\u0259rind\u0259 ba\u015f ver\u0259 bil\u0259r. Da\u015f\u0131y\u0131c\u0131 qad\u0131nlar\u0131n 50 faiz u\u015faq sahibi olma \u015fans\u0131 var, bu geni ötürürl\u0259r v\u0259 h\u0259tta t\u0259k bir x\u0259st\u0259 geni da\u015f\u0131yan o\u011flanda hemofiliya inki\u015faf edir hemofiliya v\u0259 da\u015f\u0131y\u0131c\u0131 qad\u0131n da hemofiliya inki\u015faf etdir\u0259 bil\u0259r.<\/p>\n

GENET\u0130K D\u0130AQNOST\u0130K TESTL\u018fR \u018fH\u018fM\u0130YY\u018fTL\u0130D\u0130R
\nHemofiliyan\u0131n müalic\u0259si haqq\u0131nda da m\u0259lumat ver\u0259n Prof. Dr. Köro\u011flu, "Qohum evlilikl\u0259rinin çox oldu\u011fu b\u0259zi c\u0259miyy\u0259tl\u0259rd\u0259 hemofiliya daha çox mü\u015fahid\u0259 oluna bil\u0259r. Bel\u0259 hallarda genetik diaqnostika testl\u0259rinin apar\u0131lmas\u0131, antenatal (prenatal) müayin\u0259l\u0259rl\u0259 dölün diaqnozunun qoyulmas\u0131 v\u0259 genetik m\u0259sl\u0259h\u0259tl\u0259rin verilm\u0259si tövsiy\u0259 olunur. Ancaq n\u0259 zaman hemofiliyal\u0131 körp\u0259 do\u011fulsa, ail\u0259 maarifl\u0259ndirilm\u0259li v\u0259 u\u015fa\u011fa laz\u0131mi m\u0259lumat verilm\u0259lidir (müalic\u0259d\u0259ki ç\u0259tinlikl\u0259r\u0259 gör\u0259 ilk seçimimiz hemofiliyan\u0131n qar\u015f\u0131s\u0131n\u0131n al\u0131nmas\u0131d\u0131r). \u018fv\u0259zedici müalic\u0259 yaln\u0131z venadaxili d\u0259rman enjeksiyonu il\u0259 edil\u0259 bil\u0259r. Bunun xaricind\u0259 adekvat faktorun d\u0259yi\u015fdirilm\u0259si hallar\u0131nda bel\u0259, qanaxma riski v\u0259 immun sisteminin laxtalanma faktorlar\u0131na qar\u015f\u0131 reaksiyas\u0131 bizim ilk seçimimizdir Müalic\u0259d\u0259ki ç\u0259tinlikl\u0259r" dedi.<\/p>\n","protected":false},"excerpt":{"rendered":"

17 aprel Ümumdünya Hemofiliya Günüdür. Hemofiliya, laxtalanma faktorlar\u0131n\u0131n çat\u0131\u015fmazl\u0131\u011f\u0131 s\u0259b\u0259bind\u0259n qan\u0131n laxtalana bilm\u0259diyi nadir bir x\u0259st\u0259likdir. Da\u015f\u0131y\u0131c\u0131 qad\u0131nlar\u0131n geni u\u015faqlar\u0131na ötürm\u0259 \u015fans\u0131 50 faizdir.  Ucnoqta.az x\u0259b\u0259r verir ki, hematologiya müt\u0259x\u0259ssisi…<\/p>\n","protected":false},"author":1,"featured_media":251278,"comment_status":"open","ping_status":"open","sticky":false,"template":"","format":"standard","meta":{"_lmt_disableupdate":"","_lmt_disable":"","footnotes":""},"categories":[108],"tags":[],"class_list":["post-200393","post","type-post","status-publish","format-standard","has-post-thumbnail","hentry","category-leykoz"],"_links":{"self":[{"href":"https:\/\/ucnoqta.az\/index.php?rest_route=\/wp\/v2\/posts\/200393","targetHints":{"allow":["GET"]}}],"collection":[{"href":"https:\/\/ucnoqta.az\/index.php?rest_route=\/wp\/v2\/posts"}],"about":[{"href":"https:\/\/ucnoqta.az\/index.php?rest_route=\/wp\/v2\/types\/post"}],"author":[{"embeddable":true,"href":"https:\/\/ucnoqta.az\/index.php?rest_route=\/wp\/v2\/users\/1"}],"replies":[{"embeddable":true,"href":"https:\/\/ucnoqta.az\/index.php?rest_route=%2Fwp%2Fv2%2Fcomments&post=200393"}],"version-history":[{"count":0,"href":"https:\/\/ucnoqta.az\/index.php?rest_route=\/wp\/v2\/posts\/200393\/revisions"}],"wp:featuredmedia":[{"embeddable":true,"href":"https:\/\/ucnoqta.az\/index.php?rest_route=\/wp\/v2\/media\/251278"}],"wp:attachment":[{"href":"https:\/\/ucnoqta.az\/index.php?rest_route=%2Fwp%2Fv2%2Fmedia&parent=200393"}],"wp:term":[{"taxonomy":"category","embeddable":true,"href":"https:\/\/ucnoqta.az\/index.php?rest_route=%2Fwp%2Fv2%2Fcategories&post=200393"},{"taxonomy":"post_tag","embeddable":true,"href":"https:\/\/ucnoqta.az\/index.php?rest_route=%2Fwp%2Fv2%2Ftags&post=200393"}],"curies":[{"name":"wp","href":"https:\/\/api.w.org\/{rel}","templated":true}]}}