{"id":162539,"date":"2022-11-14T11:16:07","date_gmt":"2022-11-14T07:16:07","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=162539","title":{"rendered":"Az\u0259rbaycanda \u0259sas\u0259n betta talassemiya \u00e7ox yay\u0131l\u0131b"},"content":{"rendered":"

Talessemiya valideyinl\u0259rd\u0259n genetik olaraq keç\u0259n, qanda hemaqlobin adl\u0131 madd\u0259nin yaranmas\u0131na t\u0259sir ed\u0259n genetik bir x\u0259st\u0259likdir. O, iki sözün birl\u0259\u015fm\u0259sind\u0259n \u0259m\u0259l\u0259 g\u0259lib. Talas-d\u0259niz, miya is\u0259 qan dem\u0259kdir. \u018fn çox da Asiya, Yax\u0131n \u015e\u0259rq ölk\u0259l\u0259rind\u0259, Afrikada v\u0259 Aral\u0131q d\u0259nizin sahilind\u0259 yerl\u0259\u015f\u0259n ölk\u0259l\u0259rd\u0259 rast g\u0259linir. <\/strong><\/p>\n

Az\u0259rbaycanda da talassemiya (\u0259sas\u0259n betta talassemiya) çox yay\u0131l\u0131b. Az\u0259rbaycanda β-talassemiyan\u0131n yay\u0131lma tezliyinin 3,7-8,6%, α talassemiyan\u0131n is\u0259 Q\u0259b\u0259l\u0259, \u015e\u0259ki v\u0259 Bak\u0131da müvafiq olaraq 14%, 9% v\u0259 5% olmas\u0131 mü\u0259yy\u0259n edilmi\u015fdir.<\/p>\n

Milli Hematologiya v\u0259 Transfuziologiya M\u0259rk\u0259zinin hematoloqu \u0130rad\u0259 S\u0259m\u0259d-zad\u0259 Ucnoqta.az-a bildirdi ki, x\u0259st\u0259lik hemoqlobinin sintezinin pozulmas\u0131 il\u0259 mü\u015fahid\u0259 olunan v\u0259 xroniki anemiya il\u0259 xarakteriz\u0259 olunan x\u0259st\u0259likdir. X\u0259st\u0259lik anadan g\u0259lm\u0259dir. Onun sözl\u0259rin\u0259 gör\u0259, hemoqlobin orqanizmi d\u0259mirl\u0259 t\u0259min ed\u0259n q\u0131rm\u0131z\u0131 qan hüceyr\u0259l\u0259rind\u0259n (eritrositl\u0259r) ibar\u0259t zülald\u0131r. O “hem” v\u0259 “qlobin” hiss\u0259d\u0259n ibar\u0259tdir. Hem d\u0259mir, qlobin is\u0259 zülal hiss\u0259dir. Hemoqlobinin t\u0259rkibind\u0259 bir molekul qlobin v\u0259 4 molekul hem vard\u0131r. Hemin t\u0259rkibind\u0259 olan d\u0259mir atomu oksigen molekulunu birl\u0259\u015fdirm\u0259k v\u0259 azad etm\u0259k qabiliyy\u0259tin\u0259 malikdir. \u018fsas v\u0259zif\u0259si d\u0259 t\u0259n\u0259ffüs zaman\u0131 oksigen molekullar\u0131 il\u0259 birl\u0259\u015fm\u0259k v\u0259 sonra qan dövran\u0131 il\u0259 birlikd\u0259 onlar\u0131 orqan v\u0259 toxumalara da\u015f\u0131y\u0131b verm\u0259kdir. Oksigeni verdikd\u0259n sonra hemoqlobin ba\u015fqa bir “s\u0259rni\u015fini” – karbon dioksidi götürür v\u0259 onu a\u011f ciy\u0259rl\u0259r\u0259 ötürür, oradan is\u0259 o, hava il\u0259 orqanizmd\u0259n ç\u0131x\u0131r. Bel\u0259 ç\u0131x\u0131r ki, n\u0259f\u0259s ald\u0131qda oksigen al\u0131r\u0131q, n\u0259f\u0259s verdikd\u0259 is\u0259 – karbon qaz\u0131ndan azad oluruq. Hemoqlobin olmadan bu sad\u0259c\u0259 mümkün deyil. Talassemiya x\u0259st\u0259liyin\u0259 s\u0259b\u0259bi is\u0259 orqanizmd\u0259 anormal hemoqlobin formas\u0131n\u0131n hasil edilm\u0259sidir. Yetkinl\u0259rd\u0259 hemoqlobin 4 elementin birl\u0259\u015fm\u0259sind\u0259n yaran\u0131r. Onlarda dominant hemoqlobin -hemoqlobin A (97 faiz) 2 alfa v\u0259 2 beta z\u0259ncirl\u0259rind\u0259n ibar\u0259tdir. Normal qan\u0131n kiçik bir hiss\u0259sini t\u0259\u015fkil ed\u0259n 2 alfa v\u0259 2 qamma-z\u0259ncirl\u0259rd\u0259n ibar\u0259t olan hemoqlobin F (fetal) v\u0259 2 alfa v\u0259 2 delta z\u0259ncirl\u0259rind\u0259n ibar\u0259t olan hemoqlobin A2 adlan\u0131r”.<\/p>\n

Milli Hematologiya v\u0259 Transfuziologiya M\u0259rk\u0259zinin hematoloqu \u0130rad\u0259 S\u0259m\u0259d-zad\u0259 bildirdi ki, hemoqlobin zülal\u0131na aid olan informasiya gend\u0259 da\u015f\u0131n\u0131r. “Orqanizmd\u0259 ba\u015f ver\u0259n bütün prosesl\u0259r gen t\u0259r\u0259find\u0259n kodla\u015fd\u0131r\u0131l\u0131r, onlar t\u0259r\u0259find\u0259n idar\u0259 olunur. Alfa z\u0259nciri 16-c\u0131 gend\u0259, beta z\u0259nciri is\u0259 11-ci gend\u0259 yerl\u0259\u015fir. El\u0259 talassemiya x\u0259st\u0259liyi d\u0259 h\u0259min genomun pozulmas\u0131, düzgün i\u015fl\u0259m\u0259m\u0259si n\u0259tic\u0259sind\u0259 yaran\u0131r. Alfa z\u0259ncirinin sintezinin pozulmas\u0131 alfa-talassemiya, beta z\u0259ncirinin sintezinin pozulmas\u0131 beta-talassemiya kimi tan\u0131nan klinik x\u0259st\u0259liy\u0259 s\u0259b\u0259b olur”.<\/p>\n

\u0130rad\u0259 S\u0259m\u0259d-zad\u0259 vur\u011fulad\u0131 ki, mutasiya olunmu\u015f genl\u0259rin valideynl\u0259rd\u0259n u\u015fa\u011fa ötürülm\u0259sind\u0259n as\u0131l\u0131 olaraq h\u0259min x\u0259st\u0259liyin yüngül, orta v\u0259 ya a\u011f\u0131r formas\u0131 ola bil\u0259r. “H\u0259r iki valideyind\u0259n x\u0259st\u0259 gen keç\u0259rs\u0259, t\u0259bii ki, x\u0259st\u0259lik daha a\u011f\u0131r formada ozünü göst\u0259r\u0259c\u0259k. \u018fg\u0259r yaln\u0131z bir valideynd\u0259n keç\u0259rs\u0259, bu zaman x\u0259st\u0259lik bir az yüngül \u015f\u0259kild\u0259 ortaya ç\u0131xacaq. Böyük beta talassemiya çox a\u011f\u0131rd\u0131r. \u0130lk klinik sindromlar\u0131 u\u015faq do\u011fulandan 6 ay sonra özünü göst\u0259rir. U\u015faqlar inki\u015fafdan qal\u0131r, sümükl\u0259rind\u0259 deformasiya yaran\u0131r, qan çatm\u0131r, sümük iliyi intensiv i\u015fl\u0259diyind\u0259n deformasiyaya u\u011fray\u0131r. M\u0259s\u0259l\u0259n almac\u0131q sümükl\u0259ri qabar\u0131q olur. Mikroelementl\u0259rin çat\u0131\u015fmazl\u0131\u011f\u0131 n\u0259tic\u0259sind\u0259 al\u0131n sümükl\u0259ri yast\u0131la\u015f\u0131r. Bu da u\u015faqlarda sonradan kompleksl\u0259r\u0259, psixoloji pozuntulara g\u0259tirib ç\u0131xar\u0131r”.<\/p>\n

Onun sözl\u0259rin\u0259 gör\u0259, talassemiya xroniki anemiya il\u0259 xarakteriz\u0259 olunursa, sar\u0131l\u0131q, daxili orqanlar\u0131n – dala\u011f\u0131n, qaraciy\u0259rin böyüm\u0259si ba\u015f verir. “ Xroniki anemiya qanda kifay\u0259t q\u0259d\u0259r sa\u011flam q\u0131rm\u0131z\u0131 qan hüceyr\u0259si v\u0259 ya hemoqlobin olmad\u0131\u011f\u0131 zaman meydana g\u0259lir, Hemoqlobin oksigenin da\u015f\u0131nmas\u0131ndan m\u0259sul olan q\u0131rm\u0131z\u0131 qan hüceyr\u0259l\u0259rind\u0259 olan bir molekuldur. Onun istehsal\u0131 üçün d\u0259mir z\u0259ruri komponentdir. \u0130nsan orqanizmind\u0259 olan d\u0259mirin yar\u0131dan çoxu qan hemoqlobinin t\u0259rkibind\u0259dir. Sad\u0259 dild\u0259 des\u0259k, d\u0259mir qan t\u0259rkibinin \u0259n vacib madd\u0259l\u0259rind\u0259n biridir. \u018fg\u0259r orqanizmd\u0259 d\u0259mir çat\u0131\u015fmazl\u0131\u011f\u0131 varsa bu qan\u0131n q\u0131rm\u0131z\u0131 hüceyr\u0259l\u0259rinin az olmas\u0131 dem\u0259kdir. Bel\u0259 olan halda qanda hemoqlobinin s\u0259viyy\u0259si a\u015fa\u011f\u0131 dü\u015für v\u0259 toxumalar laz\u0131m\u0131nca oksigenl\u0259 t\u0259min oluna bilmir. Qan azl\u0131\u011f\u0131 anemiya yaran\u0131r. Bu x\u0259st\u0259likl\u0259r aras\u0131nda \u0259n geni\u015f yay\u0131lan\u0131d\u0131r v\u0259 dünyan\u0131n ¼ hiss\u0259si bu narahatl\u0131qdan \u0259ziyy\u0259t ç\u0259kir. Bel\u0259likl\u0259, hemoqlobin patolojidirs\u0259, normal deyils\u0259, x\u0259st\u0259l\u0259rd\u0259 beta z\u0259ncirinin sintezi pozulubsa, anormal gen sintez olunursa, o öz funksiyas\u0131n\u0131 normal \u015f\u0259kild\u0259 yerin\u0259 yetir\u0259 bilmir v\u0259 o parçalan\u0131r. Parçalanma is\u0259 dalaqda ba\u015f verir. Dalaqda bu proses yüks\u0259k tezlikd\u0259 ba\u015f verdiyin\u0259 gör\u0259, o da böyüyür, g\u0259rgin i\u015fl\u0259diyin\u0259 gör\u0259 onun ölçül\u0259ri art\u0131r. Bu x\u0259st\u0259l\u0259r u\u015faq ya\u015flar\u0131ndan müvafiq müalic\u0259 almasalar, hemotoloqlar t\u0259r\u0259find\u0259n müalic\u0259 almasalar, 10 ya\u015f\u0131nad\u0259k t\u0259l\u0259f ola bil\u0259rl\u0259r. X\u0259st\u0259liyin m\u0259xzi d\u0259 bundan ibar\u0259tdir”.<\/p>\n

\u0130rad\u0259 S\u0259m\u0259d-zad\u0259 qeyd etdi ki, hemoqlobinin sintezinin pozulmas\u0131 orqan v\u0259 toxumalara qan t\u0259r\u0259find\u0259n oksigen v\u0259 qida madd\u0259l\u0259rinin çatd\u0131r\u0131lmas\u0131n\u0131n pozulmas\u0131na s\u0259b\u0259b olur. “Yuxar\u0131da qeyd etdiyim kimi, bundan da anemiya, sar\u0131l\u0131q qaynaqlan\u0131r. Bundan ba\u015fqa, qan\u0131n parçalanmas\u0131 n\u0259tic\u0259sind\u0259 d\u0259mir d\u0259 xaric olunur. Bu da d\u0259mir yükl\u0259nm\u0259sin\u0259 g\u0259tirib ç\u0131xard\u0131r. Eyni zamanda, x\u0259st\u0259l\u0259r münt\u0259z\u0259m transfusiya ald\u0131\u011f\u0131na gör\u0259 qan\u0131n d\u0259mir yükl\u0259nm\u0259sini a\u011f\u0131rla\u015fd\u0131r\u0131r. Bel\u0259likl\u0259, d\u0259mir yükl\u0259nm\u0259si h\u0259m ür\u0259k-damar sistemini z\u0259ifl\u0259dir, qara ciy\u0259r, h\u0259m d\u0259 böyr\u0259k sisteminin z\u0259d\u0259l\u0259nm\u0259sin\u0259 g\u0259tirib ç\u0131xar\u0131r.<\/p>\n

Bu probleml\u0259rd\u0259n \u0259ziyy\u0259t ç\u0259k\u0259n x\u0259st\u0259 u\u015faqlar\u0131n do\u011fuldu\u011fu ail\u0259l\u0259rd\u0259 t\u0259bii ki, maarifl\u0259ndirilm\u0259li i\u015fl\u0259ri apar\u0131lmal\u0131, x\u0259st\u0259 u\u015faqlara xüsusi diqq\u0259t göst\u0259rilm\u0259lidir. H. \u018fliyev fondunun t\u0259\u015f\u0259bbüsü il\u0259 Talassemiya m\u0259rk\u0259zi yarad\u0131l\u0131b. Bu m\u0259rk\u0259z aç\u0131lan günd\u0259n u\u015faqlar\u0131n müalic\u0259si kompleks \u015f\u0259kild\u0259 apar\u0131l\u0131r. H\u0259m qan t\u0259chizat\u0131 yax\u015f\u0131la\u015f\u0131b, h\u0259m d\u0259 d\u0259mir yükl\u0259nm\u0259sinin qar\u015f\u0131s\u0131n\u0131n al\u0131nmas\u0131 üçün müvafiq d\u0259rmanlar var. M\u0259rk\u0259z x\u0259st\u0259l\u0259rd\u0259 d\u0259mir yükl\u0259nm\u0259sinin qar\u015f\u0131s\u0131n\u0131 alma\u011fa köm\u0259k edir”.<\/p>\n

Pünhan \u018ff\u0259ndiyev<\/strong><\/em><\/p>\n

Yaz\u0131 "Leykozlu U\u015faqlara D\u0259st\u0259k" ictimai birliyinin Az\u0259rbaycan Respublikas\u0131n\u0131n Qeyri-Hökum\u0259t T\u0259\u015fkilatlar\u0131na Dövl\u0259t D\u0259st\u0259yi Agentliyinin maliyy\u0259 yard\u0131m\u0131 il\u0259 “Regionlarda talassemiya v\u0259 qan donorlu\u011fu bar\u0259d\u0259 maarifl\u0259ndirm\u0259” layih\u0259si ç\u0259rçiv\u0259sind\u0259 haz\u0131rlanm\u0131\u015fd\u0131r.<\/strong><\/em><\/p>\n

 <\/p>\n

 <\/p>\n","protected":false},"excerpt":{"rendered":"

Talessemiya valideyinl\u0259rd\u0259n genetik olaraq keç\u0259n, qanda hemaqlobin adl\u0131 madd\u0259nin yaranmas\u0131na t\u0259sir ed\u0259n genetik bir x\u0259st\u0259likdir. O, iki sözün birl\u0259\u015fm\u0259sind\u0259n \u0259m\u0259l\u0259 g\u0259lib. Talas-d\u0259niz, miya is\u0259 qan dem\u0259kdir. \u018fn çox da Asiya,…<\/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-162539","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\/162539","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=162539"}],"version-history":[{"count":0,"href":"https:\/\/ucnoqta.az\/index.php?rest_route=\/wp\/v2\/posts\/162539\/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=162539"}],"wp:term":[{"taxonomy":"category","embeddable":true,"href":"https:\/\/ucnoqta.az\/index.php?rest_route=%2Fwp%2Fv2%2Fcategories&post=162539"},{"taxonomy":"post_tag","embeddable":true,"href":"https:\/\/ucnoqta.az\/index.php?rest_route=%2Fwp%2Fv2%2Ftags&post=162539"}],"curies":[{"name":"wp","href":"https:\/\/api.w.org\/{rel}","templated":true}]}}