{"id":10509,"date":"2021-04-08T14:33:08","date_gmt":"2021-04-08T11:33:08","guid":{"rendered":"https:\/\/blog.annebebekkulubu.com\/?p=10509"},"modified":"2021-04-08T14:36:52","modified_gmt":"2021-04-08T11:36:52","slug":"cocukluk-caginda-ailevi-akdeniz-atesi-fmf-belirtileri-ve-tedavisi","status":"publish","type":"post","link":"https:\/\/annebebekkulubu.com\/blog\/cocukluk-caginda-ailevi-akdeniz-atesi-fmf-belirtileri-ve-tedavisi\/","title":{"rendered":"\u00c7ocukluk \u00e7a\u011f\u0131nda Ailevi Akdeniz Ate\u015fi (FMF) belirtileri ve tedavisi"},"content":{"rendered":"

Ailevi Akdeniz Ate\u015fi, \u00e7ocuklarda ataklar halinde kar\u0131n a\u011fr\u0131s\u0131 ve ate\u015f \u015fikayetleri ile kendini belli eden bir hastal\u0131kt\u0131r. Bu hastal\u0131k s\u0131radan bir ate\u015flenme durumu de\u011fildir ve tipik bir genetik hastal\u0131kt\u0131r. Ailevi Akdeniz Ate\u015fi (FMF) kimlerde g\u00f6r\u00fcl\u00fcr, belirtileri nelerdir?<\/strong> Ailevi Akdeniz Ate\u015fi tan\u0131s\u0131 nas\u0131l konur, tedavide nas\u0131l bir yol izlenir? \u00c7ocuk Sa\u011fl\u0131\u011f\u0131 ve Hastal\u0131klar\u0131 Uzman\u0131 Do\u00e7. Dr. Fatih Battal,<\/strong> \u00e7ocuklarda Ailevi Akdeniz Ate\u015fi (FMF) ile ilgili t\u00fcm merak edilenleri detaylar\u0131yla anlatt\u0131.<\/p>\n

Ailesel Akdeniz Ate\u015fi (FMF); \u00fclkemizde s\u0131k g\u00f6r\u00fclen genetik ve tekrarlayan ataklar ile karakterizedir. \u00c7ocukluk d\u00f6neminde genellikle semptomlar\u0131n g\u00f6r\u00fclmeye ba\u015flad\u0131\u011f\u0131 bir hastal\u0131kt\u0131r. T\u00fcrkiye\u2019de yap\u0131lan bir \u00e7al\u0131\u015fmada FMF tan\u0131s\u0131 alan hastalarda belirtilerin ortalama 9,6 ya\u015f\u0131nda ba\u015flad\u0131\u011f\u0131, hastalar\u0131n ortalama 16,4 ya\u015f\u0131nda tan\u0131 ald\u0131klar\u0131 saptanm\u0131\u015ft\u0131r. FMF ataklar ile seyreden, karakteristik olarak ate\u015f ve ate\u015fe e\u015flik eden kar\u0131n a\u011fr\u0131s\u0131, g\u00f6\u011f\u00fcs a\u011fr\u0131s\u0131, eklem a\u011fr\u0131s\u0131 gibi bulgularla kar\u015f\u0131m\u0131za \u00e7\u0131kar.<\/p>\n

\"\"\u00c7ocuk Sa\u011fl\u0131\u011f\u0131 ve Hastal\u0131klar\u0131 Uzman\u0131 Do\u00e7. Dr. Fatih Battal<\/em><\/p>\n

Ailevi Akdeniz Ate\u015fi (FMF) ata\u011f\u0131 nas\u0131l ba\u015flar?<\/strong><\/p>\n

Atak s\u00fcresi hastadan hastaya de\u011fi\u015fkenlik g\u00f6stermektedir. Ataklar genellikle 12-96 saat aral\u0131\u011f\u0131nda s\u00fcrmektedir. Hastalar ataklar d\u0131\u015f\u0131nda asemptomatiktir. Hastalar\u0131n atak s\u0131kl\u0131\u011f\u0131 haftada bir ile y\u0131lda bire kadar de\u011fi\u015febilen periyodlarla tekrarlayabilir.<\/p>\n

Ailevi Akdeniz Ate\u015fi (FMF) belirtileri<\/strong><\/p>\n

Ailesel Akdeniz Ate\u015fi hastalar\u0131n\u0131n ataklar\u0131nda en s\u0131k kar\u015f\u0131m\u0131za \u00e7\u0131kan semptom ve bulgular\u0131 ate\u015f (%96), kar\u0131n zar\u0131 iltihab\u0131 (%91), akci\u011fer zar\u0131 iltihab\u0131 (%57), eklem iltihab\u0131 (%45) ve amiloidoz (%2)\u2019dur.<\/p>\n

Ate\u015f<\/strong><\/p>\n

Ailesel Akdeniz Ate\u015fi nedeni ile takip edilen hastalardaki en s\u0131k semptomdur. 38-40 \u00baC bulan y\u00fcksek ate\u015f on iki saat ile \u00fc\u00e7 g\u00fcn aras\u0131nda s\u00fcrebilir. Ate\u015f erken \u00e7ocuklukta FMF\u2019nin ilk ve tek bulgusu olarak saptanabilir. Ate\u015fsiz atak az say\u0131da hastada g\u00f6r\u00fcl\u00fcr. Bu olgularda ate\u015f hasta taraf\u0131ndan fark edilmeyecek kadar az y\u00fcksektir. Kol\u015fisin tedavisi alan hastalarda atak s\u0131ras\u0131nda ate\u015f g\u00f6r\u00fclmeyebilir.<\/p>\n

Kar\u0131n a\u011fr\u0131s\u0131<\/strong><\/p>\n

Kar\u0131n a\u011fr\u0131s\u0131; FMF\u2019de %90-95 s\u0131kl\u0131kla g\u00f6r\u00fclen klinik ba\u015fka bir durumdur. Kar\u0131n a\u011fr\u0131s\u0131 ani ataklar \u015feklinde \u00f6nc\u00fc bulgu olmadan ba\u015flar. FMF\u2019de a\u011fr\u0131 ba\u015flang\u0131\u00e7ta lokalize olabilir ancak k\u0131sa s\u00fcrede t\u00fcm karna yay\u0131l\u0131r ve \u015fiddeti de\u011fi\u015fir. \u00d6yle ki; hafif bir \u015fi\u015fkinlikten a\u011f\u0131r peritonit gibi farkl\u0131 formlarda kar\u015f\u0131m\u0131za \u00e7\u0131kabilir.<\/p>\n

G\u00f6\u011f\u00fcs a\u011fr\u0131s\u0131<\/strong><\/p>\n

\u00c7ocukluk \u00e7a\u011f\u0131nda g\u00f6\u011f\u00fcs a\u011fr\u0131s\u0131 FMF ataklar\u0131nda s\u0131k kar\u015f\u0131la\u015f\u0131lan di\u011fer bir klinik durumdur. FMF hastalar\u0131nda g\u00f6\u011f\u00fcs a\u011fr\u0131s\u0131n\u0131n ortalama g\u00f6r\u00fclme s\u0131kl\u0131\u011f\u0131 %25-80 aras\u0131nda de\u011fi\u015fmektedir.<\/p>\n

Amiloidoz<\/strong><\/p>\n

Amiloidozun kesin tan\u0131s\u0131 idrar tetkikinde protein\u00fcri saptanan hastalarda yap\u0131lan renal biyopsi ile konur. Amiloidoz geli\u015fiminde erken d\u00f6nemde idrarda protein\u00fcri izlenmektedir. Bu nedenle FMF\u2019li hastalarda tam idrar tetkikinin mutlaka d\u00fczenli aral\u0131klarla takip edilmesi gerekir. Protein\u00fcri tespit edilen hastalarda biyopsi ile mutlaka amiloidoz varl\u0131\u011f\u0131 ara\u015ft\u0131r\u0131lmal\u0131d\u0131r. Protein\u00fcri tablosundan \u00fcremiye ge\u00e7i\u015f s\u00fcresi 3,5-13 y\u0131l (ortalama 7 y\u0131l) aras\u0131na saptanm\u0131\u015ft\u0131r.<\/p>\n

Ailesel Akdeniz Ate\u015fi tan\u0131s\u0131 nas\u0131l konur?<\/strong><\/p>\n

Ailesel Akdeniz Ate\u015fi tan\u0131s\u0131; klinik tan\u0131 kriterleri pozitif aile \u00f6yk\u00fcs\u00fc, di\u011fer periyodik ate\u015f sendromlar\u0131n\u0131n d\u0131\u015flanmas\u0131, kol\u015fisin tedavisine yan\u0131t ve genetik ile desteklenerek konmaktad\u0131r. FMF\u2019de semptom ve klinik bulgular\u0131n\u0131n aral\u0131kl\u0131 olarak uzun s\u00fcredir ataklar \u015feklinde tekrar etmesi, ataklar aras\u0131 d\u00f6nemde semptomlar\u0131n\u0131n ve klinik bulgular\u0131n\u0131n g\u00f6r\u00fclmemesi ve laboratuvar bulgular\u0131n\u0131n normal olmas\u0131, aile \u00f6yk\u00fcs\u00fcn\u00fcn bulunmas\u0131, kol\u015fisin tedavisine hasta yan\u0131t\u0131n iyi olmas\u0131, etnik \u00f6zellikler ve FMF tan\u0131s\u0131 koymada yard\u0131mc\u0131 ipu\u00e7lar\u0131d\u0131r. MEFV geni mutasyon analizi, sadece \u015f\u00fcphelenilen hastalarda tan\u0131n\u0131n desteklenmesi i\u00e7in kullan\u0131l\u0131r. Bununla birlikte kol\u015fisin tedavisine cevap veren ancak MEFV geninde mutasyon saptanmayan hastalar da vard\u0131r.<\/p>\n

Okumal\u0131s\u0131n\u0131z: Bebek ve \u00e7ocuklarda y\u00fcksek ate\u015f nas\u0131l \u00f6l\u00e7\u00fcl\u00fcr?<\/a><\/strong><\/p>\n

Ailesel Akdeniz Ate\u015fi tedavisi nas\u0131l yap\u0131l\u0131r?<\/strong><\/p>\n

Ailesel Akdeniz Ate\u015fi tedavisinde atak s\u0131kl\u0131\u011f\u0131n\u0131 azaltan ve ayn\u0131 zamanda amiloidoz geli\u015fimini \u00f6nleyen temel tedavi se\u00e7ene\u011fi kol\u015fisindir. \u00c7ocuklarda kol\u015fisin dozu v\u00fccut a\u011f\u0131rl\u0131\u011f\u0131na ve hastan\u0131n ya\u015f\u0131na g\u00f6re d\u00fczenlenir. Sadece atak s\u0131ras\u0131nda kol\u015fisin al\u0131nd\u0131\u011f\u0131nda etkili de\u011fildir. Ana etkisi d\u00fczenli kullan\u0131ld\u0131\u011f\u0131nda g\u00f6r\u00fclmektedir. Bazen yaln\u0131zca tek bir dozun bile atlanmas\u0131 ata\u011fa sebep olabilmektedir. Kol\u015fisin tedavisi bu nedenle ya\u015fam boyu s\u00fcrmekte ve b\u00fcy\u00fck bir titizlikle uygulanmas\u0131 gerekmektedir. Kol\u015fisin, terap\u00f6tik doz aral\u0131\u011f\u0131nda g\u00fcvenli bir ila\u00e7t\u0131r. Kol\u015fisinin en s\u0131k g\u00f6r\u00fclen yan etkisi gastrointestinal sistem \u00fczerinedir; gayta k\u0131vam\u0131nda yumu\u015fama, ishal, kar\u0131n a\u011fr\u0131s\u0131 ve kar\u0131nda \u015fi\u015fkinlik g\u00f6r\u00fclebilir.<\/p>\n

\u0130shal s\u00fcresince hastan\u0131n kol\u015fisin d\u00fczeyi azalt\u0131labilir, ishal ge\u00e7ti\u011finde ise hastan\u0131n dozu kullan\u0131lan doza y\u00fckseltilebilir. Di\u011fer s\u0131k kar\u015f\u0131la\u015f\u0131lan yan etkiler ise; karaci\u011fer enzimlerinde y\u00fckseklik, myopati, n\u00f6ropati, pansitopeni ve nadiren olabilen erizipel benzeri d\u00f6k\u00fcnt\u00fcd\u00fcr. Kol\u015fisinin \u2265 6 ay boyunca maksimum tolere edilen dozda verilmesine ra\u011fmen ayda \u22651 atak ge\u00e7irmeye devam eden hastalar\u0131n kol\u015fisine yan\u0131t vermedi\u011fi veya kol\u015fisin direnci oldu\u011fu d\u00fc\u015f\u00fcn\u00fclmektedir. Uygun dozda kol\u015fisin tedavisi almas\u0131na ra\u011fmen y\u0131lda 6\u2019dan fazla tipik FMF ata\u011f\u0131 ge\u00e7iren bir hastan\u0131n kol\u015fisine diren\u00e7li oldu\u011fu kabul edilmelidir.<\/p>\n

Sonu\u00e7 olarak FMF hastal\u0131\u011f\u0131ndan \u015f\u00fcphe edilen durumlarda hasta yak\u0131n takibe al\u0131nmal\u0131d\u0131r.<\/strong> Ya\u015fad\u0131\u011f\u0131m\u0131z co\u011frafyan\u0131n hastal\u0131\u011f\u0131 olan bu hastal\u0131kta ate\u015f takviminin tutulmas\u0131n\u0131n \u00f6nemli oldu\u011funu d\u00fc\u015f\u00fcnmekteyim. Ay\u0131r\u0131c\u0131 tan\u0131 hastal\u0131klar\u0131 dikkate al\u0131narak tedavi planlanmal\u0131d\u0131r. \u00d6m\u00fcr boyu s\u00fcrecek olan tedavi verilmeden \u00f6nce tan\u0131dan emin olunmal\u0131, hasta damgalanmamal\u0131d\u0131r. Tedavi ba\u015flanan hastalar\u0131m\u0131z ya da takibe al\u0131nan hastalar\u0131m\u0131z kontrollerini aksatmamal\u0131d\u0131r.<\/p>\n","protected":false},"excerpt":{"rendered":"

Ailevi Akdeniz Ate\u015fi, \u00e7ocuklarda ataklar halinde kar\u0131n a\u011fr\u0131s\u0131 ve ate\u015f \u015fikayetleri ile kendini belli eden bir hastal\u0131kt\u0131r. Bu hastal\u0131k s\u0131radan bir ate\u015flenme durumu de\u011fildir ve tipik bir genetik hastal\u0131kt\u0131r. Ailevi Akdeniz Ate\u015fi (FMF) kimlerde g\u00f6r\u00fcl\u00fcr, belirtileri nelerdir? Ailevi Akdeniz Ate\u015fi tan\u0131s\u0131 nas\u0131l konur, tedavide nas\u0131l bir yol izlenir? \u00c7ocuk Sa\u011fl\u0131\u011f\u0131 ve Hastal\u0131klar\u0131 Uzman\u0131 Do\u00e7. Dr. […]<\/p>\n","protected":false},"author":80,"featured_media":10512,"comment_status":"open","ping_status":"closed","sticky":false,"template":"","format":"standard","meta":{"jetpack_post_was_ever_published":false,"_jetpack_newsletter_access":"","_jetpack_dont_email_post_to_subs":false,"_jetpack_newsletter_tier_id":0,"_jetpack_memberships_contains_paywalled_content":false,"_jetpack_memberships_contains_paid_content":false,"footnotes":""},"categories":[3],"tags":[],"class_list":["post-10509","post","type-post","status-publish","format-standard","has-post-thumbnail","category-cocuk"],"jetpack_sharing_enabled":true,"jetpack_featured_media_url":"https:\/\/i0.wp.com\/annebebekkulubu.com\/blog\/wp-content\/uploads\/2021\/04\/cocuk-ates.jpg?fit=1258%2C851&ssl=1","jetpack-related-posts":[],"_links":{"self":[{"href":"https:\/\/annebebekkulubu.com\/blog\/wp-json\/wp\/v2\/posts\/10509","targetHints":{"allow":["GET"]}}],"collection":[{"href":"https:\/\/annebebekkulubu.com\/blog\/wp-json\/wp\/v2\/posts"}],"about":[{"href":"https:\/\/annebebekkulubu.com\/blog\/wp-json\/wp\/v2\/types\/post"}],"author":[{"embeddable":true,"href":"https:\/\/annebebekkulubu.com\/blog\/wp-json\/wp\/v2\/users\/80"}],"replies":[{"embeddable":true,"href":"https:\/\/annebebekkulubu.com\/blog\/wp-json\/wp\/v2\/comments?post=10509"}],"version-history":[{"count":0,"href":"https:\/\/annebebekkulubu.com\/blog\/wp-json\/wp\/v2\/posts\/10509\/revisions"}],"wp:featuredmedia":[{"embeddable":true,"href":"https:\/\/annebebekkulubu.com\/blog\/wp-json\/wp\/v2\/media\/10512"}],"wp:attachment":[{"href":"https:\/\/annebebekkulubu.com\/blog\/wp-json\/wp\/v2\/media?parent=10509"}],"wp:term":[{"taxonomy":"category","embeddable":true,"href":"https:\/\/annebebekkulubu.com\/blog\/wp-json\/wp\/v2\/categories?post=10509"},{"taxonomy":"post_tag","embeddable":true,"href":"https:\/\/annebebekkulubu.com\/blog\/wp-json\/wp\/v2\/tags?post=10509"}],"curies":[{"name":"wp","href":"https:\/\/api.w.org\/{rel}","templated":true}]}}