Manavgat \u0130l\u00e7e Sa\u011fl\u0131k M\u00fcd\u00fcr\u00fc Dr. Mustafa Er, V\u00fccutta sa\u011fl\u0131kl\u0131 olarak yer alan k\u0131rm\u0131z\u0131 kan h\u00fccrelerinin \u00fcretimini etkileyen kan bozuklu\u011fu olarak tan\u0131mlanan Akdeniz anemisi olarak da bilinen Talasemi, hakk\u0131nda bilgilendirme yapt\u0131.<\/p>\n\n\n\n
Talasemi, genellikle Akdeniz \u00fclkelerindeki \u0131rklarda g\u00f6r\u00fclen, do\u011facak \u00e7ocu\u011fa anne ve babas\u0131ndan “Beta Talasemi” geninin ge\u00e7i\u015fi ile olu\u015fan bir \u00e7e\u015fit kal\u0131tsal hastal\u0131k t\u00fcr\u00fcd\u00fcr. Hemoglobinin yap\u0131m\u0131ndaki bozukluk sonucu ortaya \u00e7\u0131kmaktad\u0131r. Kandaki alyuvarlar i\u00e7inde “hem” ve “globin” olmak \u00fczere 2 k\u0131s\u0131m vard\u0131r. Globin par\u00e7as\u0131 ise alfa ve beta olmak \u00fczere ikiye ayr\u0131l\u0131r. Genlerle y\u00f6netilen bu zincirlerden birisinin olmamas\u0131 veya yetersiz olmas\u0131 talasemiye yol a\u00e7maktad\u0131r.<\/p>\n\n\n\n
Ailesel ge\u00e7i\u015fi olan talasemi, son y\u0131llarda hem d\u00fcnya \u00fczerinde hem de T\u00fcrkiye’de s\u0131kl\u0131kla g\u00f6r\u00fcl\u00fcyor. Ancak pek \u00e7ok ta\u015f\u0131y\u0131c\u0131 oldu\u011fu i\u00e7in belirtileri hissetmiyor. Hem anne hem de baba ta\u015f\u0131y\u0131c\u0131 oldu\u011fu taktirde hastal\u0131\u011f\u0131n g\u00f6r\u00fclme riski y\u00fczde 25’lere \u00e7\u0131kabiliyor. Bug\u00fcn T\u00fcrkiye’de 1 milyon 300 bin talasemi ta\u015f\u0131y\u0131c\u0131s\u0131, T\u0131p dilinde “talasemi” olarak da ge\u00e7en Akdeniz anemisi, daha \u00e7ok Akdeniz \u00fclkelerinde g\u00f6r\u00fcld\u00fc\u011f\u00fc i\u00e7in bu isimle an\u0131l\u0131yor. Ancak hastal\u0131\u011f\u0131n etkilerini art\u0131k sadece Akdeniz \u00fclkelerinde de\u011fil d\u00fcnyan\u0131n pek \u00e7ok b\u00f6lgesinde tespit edebilAkdeniz Anemisi (Talasemi) Belirtileri Nelerdir?<\/p>\n\n\n\n
En s\u0131k g\u00f6r\u00fclen Ailesel Akdeniz Anemisi (AAA) veya t\u0131ptaki ad\u0131yla talasemi belirtileri:<\/p>\n\n\n\n
Halsizlik,<\/p>\n\n\n\n
G\u00f6zlerde sar\u0131l\u0131k,<\/p>\n\n\n\n
Dalak b\u00fcy\u00fcmesi,<\/p>\n\n\n\n
Y\u00fczdeki kemiklerde \u015fekil bozuklu\u011fu,<\/p>\n\n\n\n
Solgunluk,<\/p>\n\n\n\n
\u0130\u015ftahs\u0131zl\u0131k,<\/p>\n\n\n\n
\u0130drarda koyula\u015fmad\u0131r.<\/p>\n\n\n\n
Akdeniz anemisi nas\u0131l anla\u015f\u0131l\u0131r? sorusu ise hastalarda g\u00f6r\u00fclen belirtilere g\u00f6re farkl\u0131 \u015fekillerde cevaplanabilir. Bacak b\u00f6lgelerinde g\u00f6r\u00fclen kal\u0131c\u0131 yaralar, \u00e7abuk yorulma hissi, deri renginde koyula\u015fma gibi belirtiler hastal\u0131\u011f\u0131n ilk sinyallerini g\u00f6sterir. Talasemideki \u015fikayetler, di\u011fer demir eksikli\u011fi ve vitamin eksikli\u011fi anemisi belirtileri ile farkl\u0131l\u0131k g\u00f6sterebilir. \u00c7\u00fcnk\u00fc Akdeniz anemisi, a\u011f\u0131r bir kan hastal\u0131\u011f\u0131d\u0131r. Dolay\u0131s\u0131yla hastaya devaml\u0131 kan naklinin yap\u0131lmas\u0131 gerekebilir.<\/p>\n\n\n\n
Talasemi, Akdeniz b\u00f6lgelerinde g\u00f6r\u00fclen di\u011fer bir hastal\u0131k olan Ailesel Akdeniz Ate\u015fi (AAA) veya t\u0131ptaki ad\u0131yla FMF hastal\u0131\u011f\u0131 ile kar\u0131\u015ft\u0131r\u0131lmamal\u0131d\u0131r.<\/p>\n\n\n\n
Akdeniz Anemisi Nedenleri Nelerdir?<\/p>\n\n\n\n
Talaseminin en yayg\u0131n nedeni bozuk hemoglobin molek\u00fclleridir. Anneden veya babadan aktar\u0131lan bozuk genler, \u00e7ocuklarda genellikle Akdeniz anemisi rahats\u0131zl\u0131\u011f\u0131na neden olur. Kandaki alyuvarlar i\u00e7inde “hem” ve “globin” olmak \u00fczere 2 k\u0131s\u0131m vard\u0131r. Globin par\u00e7as\u0131 ise alfa ve beta olmak \u00fczere ikiye ayr\u0131l\u0131r. Genlerle y\u00f6netilen bu zincirlerden birisinin olmamas\u0131 veya yetersiz olmas\u0131 durumunda talasemi hastal\u0131\u011f\u0131 meydana gelebilir.<\/p>\n\n\n\n
Akdeniz Anemisi T\u00fcrleri Nelerdir?<\/p>\n\n\n\n
Talasemi min\u00f6r, talasemi maj\u00f6r ve talasemi intermedia Akdeniz anemisi t\u00fcrleridir. Hastal\u0131\u011f\u0131n t\u00fcr\u00fc, klinik ortam\u0131nda yap\u0131lan \u00e7e\u015fitli tetikler sonucu belirlenir.<\/p>\n\n\n\n
Talasemi Min\u00f6r: Akdeniz anemisi ta\u015f\u0131y\u0131c\u0131l\u0131\u011f\u0131, talasemi min\u00f6r hastalar\u0131nda g\u00f6r\u00fcl\u00fcr. Beta talasemi olarak da bilinen bu hastal\u0131k t\u00fcr\u00fcnde hastalar g\u00fcndelik ya\u015famlar\u0131na rahatl\u0131kla devam ederler. Yani hastal\u0131\u011f\u0131n hi\u00e7 bir belirtisi hissedilmez. Sadece do\u011facak \u00e7ocuklara bozuk gen aktar\u0131m\u0131 s\u00f6z konusu olabilir. Anemi, sadece evlenmeden \u00f6nce yap\u0131lan kan testlerinden \u00f6\u011frenilebilir. Bireylerde hafif yorgunluk hissi vard\u0131r. Ancak bu durum direkt talasemi ile ba\u011fda\u015ft\u0131r\u0131lmaz. Talasemi min\u00f6r testleri, mutlaka hemoglobin molek\u00fcllerine elektrik y\u00fcklenerek yap\u0131lmas\u0131 gereklidir. Aksi taktirde hastal\u0131k demir eksikli\u011fi anemisi ile kar\u0131\u015ft\u0131r\u0131larak yanl\u0131\u015f tan\u0131 konulmas\u0131na sebebiyet verebilir.<\/p>\n\n\n\n
Talasemi Maj\u00f6r: Akdeniz anemisi rahats\u0131zl\u0131\u011f\u0131n\u0131n en a\u011f\u0131r seyreden t\u00fcr\u00fcd\u00fcr. Ta\u015f\u0131y\u0131c\u0131 anne ve babadan bozuk gen aktar\u0131m\u0131 sonucu do\u011fan \u00e7ocuklarda g\u00f6r\u00fcl\u00fcr. Bebek hen\u00fcz 6 ayl\u0131kken belirtiler g\u00f6r\u00fclmeye ba\u015flar. Derin kans\u0131zl\u0131k, geli\u015fme gerili\u011fi, sar\u0131l\u0131k ba\u015fl\u0131ca belirtileri aras\u0131ndad\u0131r. Tedavi edilmedi\u011fi taktirde kalp yetmezli\u011fine ve hatta \u00f6l\u00fcme kadar giden k\u00f6t\u00fc sonu\u00e7lar do\u011furabilir.<\/p>\n\n\n\n
Talasemi hemoglobin elektroforezi (talasemi testi) gibi \u00e7ok basit ve kolay kan testleri ile konulabilmektedir.<\/p>\n\n\n\n
Talasemi Ne Zaman Belirti G\u00f6sterir?<\/p>\n\n\n\n
E\u011fer \u00e7ocuk talasemi major ise, talasemi hastas\u0131 olarak do\u011far ve ya\u015fam\u0131n ilk y\u0131llar\u0131nda a\u011f\u0131r kans\u0131zl\u0131k g\u00f6r\u00fcl\u00fcr. Bu \u00e7ocuklar, sa\u011flam hemoglabin yerine oksijen ta\u015f\u0131ma yetene\u011fi bozuk, farkl\u0131 bir hemoglabin \u00fcretirler. Bunun sonucu yeterli sa\u011fl\u0131kl\u0131 hemoglobin \u00fcretemi olmaz ve \u00e7ocuklarda kans\u0131zl\u0131k, geli\u015fme gerili\u011fi, dalak b\u00fcy\u00fcmesi, enfeksiyonlara yatk\u0131nl\u0131k, y\u00fcz kemiklerinde de\u011fi\u015fiklikler ve karaci\u011fer, kalp, pankreasla ilgili rahats\u0131zl\u0131klar g\u00f6r\u00fclebilir. Talasemi majorlu \u00e7ocuklara ya\u015fam boyu ayda 1 kez d\u00fczenli kan, eritrosit transf\u00fczyonu yap\u0131lmal\u0131d\u0131r. Eritrosit transf\u00fczyonlar\u0131, yan etki olarak v\u00fccutta demir birikmesine neden olur. Demir birikimi, kalp ve karaci\u011fer ba\u015fta olmak \u00fczere organlarda birikir ve organlara zarar verebilir. Bu hastalara demir birikimini azaltmak i\u00e7in 2-3 ya\u015f civar\u0131nda v\u00fccuttan demir at\u0131l\u0131m\u0131n\u0131 artt\u0131ran ila\u00e7lar uygulan\u0131r.<\/p>\n\n\n\n
Talasemi Belirtileri Nelerdir?<\/p>\n\n\n\n
Talasemi Min\u00f6r\u2019de tek bulgu kans\u0131zl\u0131kt\u0131r. Hasta halsiz, yorgundur. Talasemi Major ise genellikle do\u011fumdan itibaren kendini g\u00f6sterir. Bebek 4-6 ayl\u0131k oldu\u011funda kans\u0131zl\u0131k ve sar\u0131l\u0131k belirginle\u015fir. Hemoglobin de\u011feri d\u00fc\u015fmeye ba\u015flar. Rengi solar, i\u015ftahs\u0131zl\u0131k ve kusma g\u00f6r\u00fcl\u00fcr. Kans\u0131zl\u0131\u011f\u0131 dengeleyebilmek i\u00e7in kemik ili\u011fi alyuvarlar\u0131n yap\u0131m\u0131n\u0131 artt\u0131r, kemik ili\u011finin \u00e7ok \u00e7al\u0131\u015fmas\u0131 ve geni\u015flemesi sonucu olarak da y\u00fcz kemiklerinde de\u011fi\u015fiklikler olu\u015fur ve y\u00fcz\u00fcn g\u00f6r\u00fcn\u00fcm\u00fcn\u00fc bozulur. Demir birikimi sebebiyle de kalp, karaci\u011fer, pankreas gibi organlarda b\u00fcy\u00fcme olu\u015fabilir. Karaci\u011ferde ve dalakta b\u00fcy\u00fcmeye ba\u011fl\u0131 kar\u0131n \u015fi\u015fli\u011fi geli\u015fir. Y\u00fcz kemiklerinde bozulmalar meydana gelir. Al\u0131n, elmac\u0131k kemiklerinin d\u0131\u015fa \u00e7\u0131k\u0131kl\u0131\u011f\u0131, burun k\u00f6k\u00fc bas\u0131kl\u0131\u011f\u0131 olu\u015fur, \u00fcst di\u015fler \u00f6ne do\u011fru f\u0131rlar. Kafa d\u00f6rt k\u00f6\u015fe \u015feklini al\u0131r. Boy k\u0131sad\u0131r.<\/p>\n\n\n\n
Akdeniz Anemisi Tedavisi Nas\u0131l Yap\u0131l\u0131r?<\/p>\n\n\n\n
Talaseminin t\u00fcr\u00fcne g\u00f6re hastalara farkl\u0131 tedavi y\u00f6ntemleri uygulan\u0131r. Ancak hastalar\u0131n geneli her 3-4 haftada kan nakline ihtiya\u00e7 duyar. Hemoglobin de\u011ferlerinin 9,5 g\/dl’nin \u00fczerinde tutulmas\u0131 gereklidir.nTedavi esnas\u0131nda dikkat edilecek as\u0131l husus demir birikimidir. S\u0131kl\u0131kla kan naklinin yap\u0131lmas\u0131, hastalarda demir birikimine yol a\u00e7arak dalak, pankreas, kalp ve karaci\u011fer organlar\u0131nda h\u00fccre hasar\u0131 meydana getirebilir. Dolay\u0131s\u0131yla demir birikimini \u00f6nlemek i\u00e7in 3 ya\u015f\u0131ndan itibaren desferrioksamin ad\u0131nda deri alt\u0131 inf\u00fczyonu ile verilen bir ila\u00e7 uygulan\u0131r.<\/p>\n\n\n\n
Akdeniz Anemisi Tedavi Edilmedi\u011finde Ortaya \u00c7\u0131kabilecek Sorunlar Nelerdir?<\/p>\n\n\n\n
\u0130nce ve gevrek kemikler,<\/p>\n\n\n\n
Konjestif kalp yetmezli\u011fi,<\/p>\n\n\n\n
Premat\u00fcre bebekler (bazen bebekler anne karn\u0131nda \u00f6lebilir)<\/p>\n\n\n\n
Karaci\u011fer, dalak ve kalp b\u00fcy\u00fcmeleri,<\/p>\n\n\n\n
Ekstramed\u00fcller eritropoietik doku t\u00fcm\u00f6rleri<\/p>\n\n\n\n
B\u00fc\u015fra Deveci \/ Haber<\/p>\n","protected":false},"excerpt":{"rendered":"
Manavgat \u0130l\u00e7e Sa\u011fl\u0131k M\u00fcd\u00fcr\u00fc Dr. Mustafa Er, V\u00fccutta sa\u011fl\u0131kl\u0131 olarak yer alan k\u0131rm\u0131z\u0131 kan h\u00fccrelerinin \u00fcretimini etkileyen kan bozuklu\u011fu olarak tan\u0131mlanan Akdeniz anemisi olarak da bilinen [more…]<\/a><\/p>\n","protected":false},"author":2,"featured_media":23545,"comment_status":"open","ping_status":"open","sticky":false,"template":"","format":"standard","meta":{"footnotes":""},"categories":[2],"tags":[],"class_list":["post-23544","post","type-post","status-publish","format-standard","has-post-thumbnail","hentry","category-gundem"],"_links":{"self":[{"href":"https:\/\/akdenizhaber.com.tr\/index.php\/wp-json\/wp\/v2\/posts\/23544","targetHints":{"allow":["GET"]}}],"collection":[{"href":"https:\/\/akdenizhaber.com.tr\/index.php\/wp-json\/wp\/v2\/posts"}],"about":[{"href":"https:\/\/akdenizhaber.com.tr\/index.php\/wp-json\/wp\/v2\/types\/post"}],"author":[{"embeddable":true,"href":"https:\/\/akdenizhaber.com.tr\/index.php\/wp-json\/wp\/v2\/users\/2"}],"replies":[{"embeddable":true,"href":"https:\/\/akdenizhaber.com.tr\/index.php\/wp-json\/wp\/v2\/comments?post=23544"}],"version-history":[{"count":1,"href":"https:\/\/akdenizhaber.com.tr\/index.php\/wp-json\/wp\/v2\/posts\/23544\/revisions"}],"predecessor-version":[{"id":23546,"href":"https:\/\/akdenizhaber.com.tr\/index.php\/wp-json\/wp\/v2\/posts\/23544\/revisions\/23546"}],"wp:featuredmedia":[{"embeddable":true,"href":"https:\/\/akdenizhaber.com.tr\/index.php\/wp-json\/wp\/v2\/media\/23545"}],"wp:attachment":[{"href":"https:\/\/akdenizhaber.com.tr\/index.php\/wp-json\/wp\/v2\/media?parent=23544"}],"wp:term":[{"taxonomy":"category","embeddable":true,"href":"https:\/\/akdenizhaber.com.tr\/index.php\/wp-json\/wp\/v2\/categories?post=23544"},{"taxonomy":"post_tag","embeddable":true,"href":"https:\/\/akdenizhaber.com.tr\/index.php\/wp-json\/wp\/v2\/tags?post=23544"}],"curies":[{"name":"wp","href":"https:\/\/api.w.org\/{rel}","templated":true}]}}