{"id":12064,"date":"2022-07-17T13:11:07","date_gmt":"2022-07-17T10:11:07","guid":{"rendered":"http:\/\/blog.ulubat.org\/?p=12064"},"modified":"2022-07-17T16:11:56","modified_gmt":"2022-07-17T13:11:56","slug":"fmf-ailevi-akdeniz-atesi","status":"publish","type":"post","link":"https:\/\/blog.ulubat.org\/index.php\/genel\/fmf-ailevi-akdeniz-atesi\/","title":{"rendered":"FMF (Ailevi Akdeniz Ate\u015fi)"},"content":{"rendered":"\n

FMF (Ailevi Akdeniz Ate\u015fi) Nedir?<\/h2>\n\n\n\n

<\/p>\n\n\n\n

FMF Nedir<\/h4>\n\n\n\n

FMF genellikle otozomal resesif kal\u0131t\u0131lml\u0131 otoinflamatuar bir hastal\u0131kt\u0131r. Periyodik ate\u015fli hastal\u0131klar\u0131n en eskisi ve en yayg\u0131n\u0131d\u0131r.<\/p>\n\n\n\n

FMF Ad\u0131n\u0131n K\u00f6keni ve Alternatif Adland\u0131rmalar<\/h4>\n\n\n\n

FMF familial mediterranean fever’\u0131n k\u0131saltmas\u0131d\u0131r ki bu da T\u00fcrk\u00e7ede ailevi akdeniz ate\u015fi anlam\u0131na gelmektedir. Periyodik peritonit, ailesel paroksismal poliserozit, periyodik hastal\u0131k, Ermeni hastal\u0131\u011f\u0131, Siegal-Cattan-Mamou hastal\u0131\u011f\u0131, Wolff periyodik hastal\u0131\u011f\u0131 ya da Reimann sendromu olarak da bilinmektedir. Daha \u00e7ok Akdeniz-Orta Do\u011fu \u00e7evresinde g\u00f6r\u00fcld\u00fc\u011f\u00fc i\u00e7in FMF ismi uygun g\u00f6r\u00fclm\u00fc\u015ft\u00fcr. <\/p>\n\n\n\n

FMF’te Epidemiyoloji<\/h4>\n\n\n\n

FMF; Yahudiler, Ermeniler, Araplar, Yunanl\u0131lar, T\u00fcrkler, K\u00fcrtler, Farslar ve \u0130talyanlar dahil olmak \u00fczere Akdeniz ve Ortado\u011fu k\u00f6kenli insanlarda yayg\u0131n olarak g\u00f6r\u00fcl\u00fcr. Ermenistan’da FMF ta\u015f\u0131y\u0131c\u0131l\u0131k oran\u0131 5 sa\u011fl\u0131kl\u0131 bireyde 1 civar\u0131ndad\u0131r. T\u00fcrkiye’de yap\u0131lan saha ara\u015ft\u0131rmalar\u0131ndan biri, FMF prevalans\u0131n\u0131n 1000 \u00e7ocukta 1 civar\u0131nda oldu\u011funu g\u00f6stermi\u015ftir. \u00c7al\u0131\u015fmalar\u0131n \u00e7o\u011funa g\u00f6re, erkeklerde ve kad\u0131nlarda FMF insidans\u0131nda anlaml\u0131 bir fark yoktur.<\/p>\n\n\n\n

FMF’te Ataklar ve Semptomlar<\/h4>\n\n\n\n

FMF kendini ataklar\/n\u00f6betler ile kendini g\u00f6steren bir hastal\u0131kt\u0131r. Atak s\u0131ras\u0131nda s\u0131kl\u0131kla hastalar g\u00f6\u011f\u00fcs, kar\u0131n ve eklemlerde ciddi a\u011fr\u0131lar hisseder ve ate\u015fi \u00e7\u0131kar. Daha az yayg\u0131n olmakla birlikte d\u00f6k\u00fcnt\u00fc ve ayak, bacaklar\u0131 etkileyecek \u015fekilde efor ile artan myalji de g\u00f6r\u00fclebilmektedir. \u0130lk atak s\u0131kl\u0131kla 20 ya\u015f\u0131n alt\u0131ndayken g\u00f6r\u00fclmektedir. Ataklar 2 ile 4 saat aras\u0131nda geli\u015fir ve 6 saat ile 4 g\u00fcn aras\u0131nda devam eder. <\/p>\n\n\n\n

FMF’te Tan\u0131<\/h4>\n\n\n\n

Tan\u0131 hastan\u0131n klinik bulgular\u0131 ve aile \u00f6yk\u00fcs\u00fc g\u00f6z \u00f6n\u00fcnde bulundurarak konulur. Ataklar s\u0131ras\u0131nda kanda l\u00f6kosit, fibrinojen ve CRP de\u011feri y\u00fckselebilir. Atak sonras\u0131 bu de\u011ferlerin y\u00fckselmesi tan\u0131 koyulmas\u0131 i\u00e7in b\u00fcy\u00fck \u00f6nem ta\u015f\u0131r. Kesin tan\u0131 genetik inceleme ile yap\u0131l\u0131r. <\/p>\n\n\n\n

FMF’te Patofizyoloji<\/h4>\n\n\n\n

FMF pirini kodlayan MEFV genindeki bozukluklar sonucu olu\u015fur. MEFV mutasyonlar\u0131 pirinin yap\u0131s\u0131n\u0131 ve i\u015flevini bozar bu da IL-1 (interl\u00f6kin) Beta’n\u0131n ve ard\u0131ndan inflamatuar yolun aktivasyonuna yol a\u00e7ar. <\/p>\n\n\n\n

FMF’te Komplikasyonlar<\/h4>\n\n\n\n

Amiloidoz FMF’in en \u00f6nemli komplikasyonudur. Amiloid A birikimi organlarda inflamasyona ve sonu\u00e7 olarak hasara sebep olur. Amiloidoz gastrointestinal sistem, dalak, karaci\u011fer, testisler, kalp ve akci\u011ferleri de etkileyebilir ancak en yayg\u0131n komplikasyon renal amiloidozdur. Amiloidoz \u015f\u00fcphesi olan FMF hastalar\u0131n\u0131n b\u00f6brek biyopsilerinde glomer\u00fclonefrit bildirilmi\u015ftir. Bunlar\u0131n yan\u0131nda FMF b\u00f6breklere zarar vererek Nefrotik Sendrom geli\u015fmesine sebep olabilir. Nefrotik Sendromda s\u0131kl\u0131kla protein\u00fcri g\u00f6zlenir. Nefrotik Sendrom renal ven trombozuna ve b\u00f6brek yetmezli\u011fine yol a\u00e7abilir. Ayr\u0131ca FMF’in neden oldu\u011fu iltihaplanma \u00fcreme organlar\u0131n\u0131 etkileyerek k\u0131s\u0131rl\u0131\u011fa sebep olabilir. <\/p>\n\n\n\n

FMF’te Tedavi ve Yan Etki<\/h4>\n\n\n\n

FMF i\u00e7in g\u00fcn\u00fcm\u00fczde en etkili tedavi ve koruma y\u00f6ntemi g\u00fcnl\u00fck ve \u00f6m\u00fcr boyu kol\u015fisin kullan\u0131m\u0131d\u0131r. Kol\u015fisin n\u00f6bet geli\u015fimini ve amiloidozu engeller. Dozaj, ya\u015fa ve semptomlar\u0131n \u015fiddetine g\u00f6re de\u011fi\u015fir. Be\u015f ya\u015f\u0131n alt\u0131ndaki \u00e7ocuklara 0.03-0.07mg\/kg\/g\u00fcn kol\u015fisin verilebilir. Doz, \u00e7ocuk b\u00fcy\u00fcd\u00fck\u00e7e 0,5 mg’l\u0131k art\u0131\u015flarla art\u0131r\u0131labilir. 1 mg kol\u015fisin, on ya\u015f\u0131ndan b\u00fcy\u00fck \u00e7ocuklar ve yeti\u015fkinler i\u00e7in idealdir. S\u0131k ataklar veya amiloidoz varl\u0131\u011f\u0131 gibi daha komplike durumlar\u0131 olan hastalara, hastalar\u0131n karaci\u011fer ve b\u00f6brek fonksiyonlar\u0131 normal ise daha y\u00fcksek dozlar (2 ila 2.5 mg) verilebilir. Amiloidoza ba\u011fl\u0131 son d\u00f6nem b\u00f6brek hastal\u0131\u011f\u0131 nedeniyle b\u00f6brek nakli yap\u0131lan hastalar, nakilden sonra bile kol\u015fisin almaya devam etmelidir. Kol\u015fisin tedavisinde en yayg\u0131n yan etkiler kusma ve ishaldir, kol\u015fisinin ba\u015fka bir piyasa muadiline ge\u00e7ilmesi ile bu yan etkiler normale d\u00f6nd\u00fcr\u00fclebilir. Di\u011fer yayg\u0131n olmayan yan etkiler miyelosupresyon, hepatotoksisite, nefrotoksisite, miyopati, n\u00f6ropati ve a\u015f\u0131r\u0131 duyarl\u0131l\u0131k reaksiyonudur. Ayr\u0131ca kol\u015fisin hamilelik ve emzirme d\u00f6neminde g\u00fcvenli kabul edilir. Kimi hastalarda kol\u015fisine diren\u00e7 g\u00f6zlenebilir bu gibi durumlarda tam olarak etkinli\u011fi kan\u0131tlanmam\u0131\u015f olsa da IL-1 inhibit\u00f6rleri kullan\u0131lmaktad\u0131r.<\/p>\n\n\n

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https:\/\/www.flickr.com\/photos\/mzuckerm\/7980626003\/<\/figcaption><\/figure><\/div>\n\n\n

<\/p>\n\n\n\n

Kol\u015fisin<\/h3>\n\n\n\n

Kol\u015fisin (colchicine); \u00f6zellikle Gut, Beh\u00e7et Hastal\u0131\u011f\u0131 ve FMF tedavisinde kullan\u0131lan bir ila\u00e7t\u0131r. G\u00fcz \u00c7i\u011fdemi (Colchicum Autumnale) \u00e7i\u00e7e\u011finden elde edildi\u011finden bu isimle an\u0131l\u0131r. \u00c7e\u015fitli romatizmal hastal\u0131klar\u0131n tedavisinde kullan\u0131ld\u0131\u011f\u0131 gibi kanser tedavisi i\u00e7in de ara\u015ft\u0131r\u0131lmaktad\u0131r. Kol\u015fisin, FMF’deki inflamatuar olaylar i\u00e7in gerekli olan n\u00f6trofil kemotaksisini inhibe ederek \u00e7al\u0131\u015f\u0131r. Kol\u015fisin, FMF’deki inflamatuar olaylar i\u00e7in gerekli olan n\u00f6trofil kemotaksisini inhibe ederek \u00e7al\u0131\u015f\u0131r.<\/p>\n\n\n

\n
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Kol\u015fisin (Colchicine)<\/mark><\/em><\/strong> https:\/\/upload.wikimedia.org\/wikipedia\/commons\/thumb\/2\/20\/Colchicine.svg\/640px-Colchicine.svg.png<\/figcaption><\/figure><\/div>\n\n\n

Yazar: Serhat Baran Arit\u00fcrk<\/p>\n\n\n\n

Edit\u00f6r: Hilal T\u00fcrkan<\/p>\n\n\n\n

<\/p>\n\n\n\n

Kaynak\u00e7a:<\/h5>\n\n\n\n

Myrna Medlej-Hashim , Jacques Loiselet, G\u00e9rard Lefranc, Andr\u00e9 M\u00e9garban\u00e9. Familial Mediterranean Fever (FMF): from diagnosis to treatment. Ekim-Aral\u0131k 2004 PMID: 15745878 <\/p>\n\n\n\n

Huri Ozdogan , Serdal Ugurlu. Familial Mediterranean Fever. \u015eubat 2019 PMID: 30686512<\/p>\n\n\n\n

Fatos Onen. Familial Mediterranean Fever. Nisan 2006 PMID: 16283319<\/p>\n\n\n\n

Kiyoshi Migita , Tomoyuki Asano , Shuzo Sato , Tomohiro Koga , Yuya Fujita , Atsushi Kawakami. Familial Mediterranean fever: overview of pathogenesis, clinical features and management. Haziran 2018 PMID: 30938266<\/p>\n\n\n\n

Ben-Chetrit E, Touitou I. Familial mediterranean Fever in the world. Ekim 2009 PMID: 19790133<\/p>\n\n\n\n

Sohar E, Gafni J, Pras M, Heller H. Familial Mediterranean fever. A survey of 470 cases and review of the literature. A\u011fustos 1967 PMID: 5340644<\/p>\n\n\n\n

Familial Mediterranean fever (FMF) in Turkey: results of a nationwide multicenter study. Ocak 2005 PMID: 15643295<\/p>\n\n\n\n

Ozen S, Karaaslan Y, Ozdemir O, Saatci U, Bakkaloglu A, Koroglu E, Tezcan S. Prevalence of juvenile chronic arthritis and familial Mediterranean fever in Turkey: a field study. Aral\u0131k 1998 PMID: 9858443<\/p>\n\n\n\n

Sarkisian T, Ajrapetian H, Beglarian A, Shahsuvarian G, Egiazarian A. Familial Mediterranean Fever in Armenian population. Mart 2008 PMID: 18403822<\/p>\n\n\n\n

Stoffman N, Magal N, Shohat T, Lotan R, Koman S, Oron A, Danon Y, Halpern GJ, Lifshitz Y, Shohat M. Higher than expected carrier rates for familial Mediterranean fever in various Jewish ethnic groups. Nisan 2000 PMID: 10854115<\/p>\n\n\n\n

https:\/\/www.ncbi.nlm.nih.gov\/books\/NBK560754\/<\/p>\n\n\n\n

https:\/\/www.trasd.org.tr\/hastalik\/ailevi-akdeniz-atesi<\/p>\n\n\n\n

http:\/\/ailehekimi.medicine.ankara.edu.tr\/wp-content\/uploads\/sites\/581\/2019\/04\/Ailevi-Akdeniz-Ate%C5%9Fi.pdf<\/p>\n\n\n\n

https:\/\/www.mayoclinic.org\/diseases-conditions\/familial-mediterranean-fever\/symptoms-causes\/syc-20372470<\/p>\n\n\n\n

https:\/\/tr.wikipedia.org\/wiki\/Ailevi_Akdeniz_ate%C5%9Fi<\/p>\n\n\n\n

<\/p>\n\n\n\n

<\/p>\n\n\n\n

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

FMF (Ailevi Akdeniz Ate\u015fi) Nedir? FMF Nedir FMF genellikle otozomal resesif kal\u0131t\u0131lml\u0131 otoinflamatuar bir hastal\u0131kt\u0131r. Periyodik ate\u015fli hastal\u0131klar\u0131n en eskisi<\/p>\n","protected":false},"author":1139,"featured_media":12079,"comment_status":"open","ping_status":"open","sticky":false,"template":"","format":"standard","meta":[],"categories":[1,2173],"tags":[2269,2268,354,73,2277,2271,2267,149,115,2276,2275,2266,2272,2270,265,84,66],"acf":[],"views":530,"_links":{"self":[{"href":"https:\/\/blog.ulubat.org\/index.php\/wp-json\/wp\/v2\/posts\/12064"}],"collection":[{"href":"https:\/\/blog.ulubat.org\/index.php\/wp-json\/wp\/v2\/posts"}],"about":[{"href":"https:\/\/blog.ulubat.org\/index.php\/wp-json\/wp\/v2\/types\/post"}],"author":[{"embeddable":true,"href":"https:\/\/blog.ulubat.org\/index.php\/wp-json\/wp\/v2\/users\/1139"}],"replies":[{"embeddable":true,"href":"https:\/\/blog.ulubat.org\/index.php\/wp-json\/wp\/v2\/comments?post=12064"}],"version-history":[{"count":10,"href":"https:\/\/blog.ulubat.org\/index.php\/wp-json\/wp\/v2\/posts\/12064\/revisions"}],"predecessor-version":[{"id":12104,"href":"https:\/\/blog.ulubat.org\/index.php\/wp-json\/wp\/v2\/posts\/12064\/revisions\/12104"}],"wp:featuredmedia":[{"embeddable":true,"href":"https:\/\/blog.ulubat.org\/index.php\/wp-json\/wp\/v2\/media\/12079"}],"wp:attachment":[{"href":"https:\/\/blog.ulubat.org\/index.php\/wp-json\/wp\/v2\/media?parent=12064"}],"wp:term":[{"taxonomy":"category","embeddable":true,"href":"https:\/\/blog.ulubat.org\/index.php\/wp-json\/wp\/v2\/categories?post=12064"},{"taxonomy":"post_tag","embeddable":true,"href":"https:\/\/blog.ulubat.org\/index.php\/wp-json\/wp\/v2\/tags?post=12064"}],"curies":[{"name":"wp","href":"https:\/\/api.w.org\/{rel}","templated":true}]}}