Akdeniz anemisi ya da tptaki ad\u0131yla Talasemi ; Akdeniz \u00fclkelerindeki \u0131rklarda (Beta-Talasemi geni nedeniyle) g\u00f6r\u00fclen. \u00e7ocu\u011fa kal\u0131t\u0131msal (otozomal resesif ) olarak ge\u00e7en bir \u00e7e\u015fit hipokrom anemidir. Hastal\u0131\u011f\u0131n de\u011fi\u015fik \u015fekilleri vard\u0131r ve bu hastal\u0131klar\u0131n a\u011f\u0131rl\u0131klar\u0131 farkl\u0131d\u0131r.
\n Anemi (kans\u0131zl\u0131k) olu\u015fmas\u0131na neden olan etmen kanda alyuvarlar\u0131n yap\u0131s\u0131nda yer alan \u201chemoglobin\u201d maddesinin yap\u0131m\u0131ndaki kusurdur.
\n T.Major ve T.Minor olarak iki formu vard\u0131r. <\/p>\n
T. Minor major forma g\u00f6re \u00e7ok daha hafif seyreder. Heterozigot formdur. Bireylerde sadece anemi vard\u0131r. Hatta baz\u0131lar\u0131 evlenme i\u015flemlerinde yap\u0131lan (zorunlu) kan testine kadar bu durumu bilmezler. Serum demir d\u00fczeyi normal veya artm\u0131\u015ft\u0131r. En \u00e7ok g\u00f6r\u00fclen anemi olan ve bu hastal\u0131kla en \u00e7ok kar\u0131\u015ft\u0131r\u0131lan Demir Eksikli\u011fi Anemisi\u2019nde ise azalm\u0131\u015ft\u0131r. Tan\u0131 Hemoglobin Elektroforezi ile konulur. HbA2 normalde %34 iken bu hastal\u0131kta % 7 ye y\u00fckselmi\u015ftir. HbF hafif d\u00fczeyde (%2-6) artm\u0131\u015ft\u0131r. T. Minor\u2019\u00fcn esas \u00f6nemi evli \u00e7iftlerin her ikisinde de bu hastal\u0131ktan olmas\u0131nda ortaya \u00e7\u0131kar; \u00e7ocu\u011fun %25 T. Major olma riski mevcuttur. <\/p>\n
Talasemi Major ise hastal\u0131\u011f\u0131n homozigot formudur ve bir di\u011fer ismi Cooley anemisidir. \u00c7o\u011funlukla bebek daha 6 ayl\u0131kken birdenbire ba\u015flayan a\u011f\u0131r anemi sonucu kalp yetmezli\u011fi geli\u015fir. Bunun olmamas\u0131 i\u00e7in d\u00fczenli olarak s\u0131k s\u0131k kan nakli yap\u0131lmal\u0131d\u0131r. Kan nakli yap\u0131lmazsa hasta birka\u00e7 senede \u00f6l\u00fcr. Kan nakli yetersiz yap\u0131l\u0131rsa kemik ili\u011finin a\u015f\u0131r\u0131 kan yapmas\u0131 sonucu harap olan kemiklerde k\u0131r\u0131lmalar olur y\u00fcz \u015fekli de\u011fi\u015fir: Burun k\u00f6k\u00fc \u00e7\u00f6k\u00fck al\u0131n ve elmac\u0131k kemikleri \u00e7\u0131k\u0131kt\u0131r. \u00dcst di\u015fler \u00f6ne f\u0131rlam\u0131\u015ft\u0131r.Ba\u015f d\u00f6rt k\u00f6\u015fe \u015feklindedir. Dalak ve karaci\u011fer b\u00fcy\u00fcr. Boy k\u0131sa kal\u0131r. \u00c7ocuk ergenlik \u00e7a\u011f\u0131na giremez.Kan nakilleriyle v\u00fccutta biriken a\u015f\u0131r\u0131 demirin yol a\u00e7t\u0131\u011f\u0131 kalp problemleri (myokardit kalp yetmezli\u011fi vs) ileriki ya\u015flarda \u00e7o\u011funlukla \u00f6l\u00fcm sebebidir. Hemoglobin elektroforezinde % 50-90 vakada g\u00f6r\u00fclen HbF tan\u0131 koydurucudur. Tedavide kan nakillerinin yan\u0131s\u0131ra v\u00fccutta biriken fazla demirin idrar at\u0131l\u0131m\u0131n\u0131 sa\u011flayan Desferoksamin ve C vitamini verilir. A\u015f\u0131r\u0131 b\u00fcy\u00fcm\u00fc\u015f dalak ameliyatla al\u0131n\u0131r. Dalak ameliyat\u0131 sonras\u0131 depo penisilin koruma (profilaktik) tedavisi ve pn\u00f6mokok a\u015f\u0131s\u0131 yap\u0131l\u0131r. \u00d6zellikle erken ya\u015fta( hen\u00fcz kan nakilleri fazlaca yap\u0131lmadan) doku tipi uyan kemik ili\u011fi nakli ile bu hastalar %70-80 tam olarak sa\u011fl\u0131klar\u0131na kavu\u015fabilmektedir. <\/p>\n
\u00dclkemizin de bir Akdeniz \u00fclkesi olmas\u0131 nedeniyle T\u00fcrkiye toplumu olarak beta-talasemi geni ta\u015f\u0131ma riskimiz vard\u0131r. Bunun i\u00e7in her \u00e7ifte evlenmeden \u00f6nce mecburi yap\u0131lan tahliller i\u00e7inde Hemoglobin (Hemogram i\u00e7inde) ve Hemoglobin Elektroforezi de yer al\u0131r.<\/p>\n","protected":false},"excerpt":{"rendered":"
Akdeniz anemisi ya da tptaki ad\u0131yla Talasemi ; Akdeniz \u00fclkelerindeki \u0131rklarda (Beta-Talasemi geni nedeniyle) g\u00f6r\u00fclen. \u00e7ocu\u011fa kal\u0131t\u0131msal (otozomal resesif ) olarak ge\u00e7en bir \u00e7e\u015fit hipokrom anemidir. Hastal\u0131\u011f\u0131n de\u011fi\u015fik \u015fekilleri vard\u0131r ve bu hastal\u0131klar\u0131n a\u011f\u0131rl\u0131klar\u0131 farkl\u0131d\u0131r. Anemi (kans\u0131zl\u0131k) olu\u015fmas\u0131na neden olan etmen kanda alyuvarlar\u0131n yap\u0131s\u0131nda yer alan \u201chemoglobin\u201d maddesinin yap\u0131m\u0131ndaki kusurdur. T.Major ve T.Minor olarak iki …<\/p>\n","protected":false},"author":1,"featured_media":0,"comment_status":"open","ping_status":"open","sticky":false,"template":"","format":"standard","meta":{"footnotes":""},"categories":[1407,1403],"tags":[6413,6409,6055,6412,6411,6410],"class_list":["post-2576","post","type-post","status-publish","format-standard","hentry","category-fen-ve-teknoloji-odevleri","category-odevler","tag-hemogram","tag-akdeniz-anemisi","tag-heterozigot","tag-myokardit-kalp-yetmezligi","tag-otozomal-resesif","tag-talasemisi"],"_links":{"self":[{"href":"https:\/\/www.islamidavet.com\/kutuphane\/wp-json\/wp\/v2\/posts\/2576","targetHints":{"allow":["GET"]}}],"collection":[{"href":"https:\/\/www.islamidavet.com\/kutuphane\/wp-json\/wp\/v2\/posts"}],"about":[{"href":"https:\/\/www.islamidavet.com\/kutuphane\/wp-json\/wp\/v2\/types\/post"}],"author":[{"embeddable":true,"href":"https:\/\/www.islamidavet.com\/kutuphane\/wp-json\/wp\/v2\/users\/1"}],"replies":[{"embeddable":true,"href":"https:\/\/www.islamidavet.com\/kutuphane\/wp-json\/wp\/v2\/comments?post=2576"}],"version-history":[{"count":0,"href":"https:\/\/www.islamidavet.com\/kutuphane\/wp-json\/wp\/v2\/posts\/2576\/revisions"}],"wp:attachment":[{"href":"https:\/\/www.islamidavet.com\/kutuphane\/wp-json\/wp\/v2\/media?parent=2576"}],"wp:term":[{"taxonomy":"category","embeddable":true,"href":"https:\/\/www.islamidavet.com\/kutuphane\/wp-json\/wp\/v2\/categories?post=2576"},{"taxonomy":"post_tag","embeddable":true,"href":"https:\/\/www.islamidavet.com\/kutuphane\/wp-json\/wp\/v2\/tags?post=2576"}],"curies":[{"name":"wp","href":"https:\/\/api.w.org\/{rel}","templated":true}]}}