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AİLESEL AKDENİZ ATEŞİ

FAMILIAL MEDITERRANEAN FEVER

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Abstract (2. Language): 
Aim: Familial Mediterranean fever, is the most common and well known disease in hereditary peri¬odic fever syndromes at the present time. In this review we summarized the disease, which was seen frequently in our country, and newly treatment methods. Main findings: Familial Mediterranean fever was described in a Jewish girl in 1908. Generally it was seen in Armenians, Sefaradian Jew, Arabians and Anatolian Turks. Pathogenesis of the disease was not clear. Characteristic feature of the disease is acute fever attacks accompanied with abdominal pain, chest pain and joint pain. The most important complication is type AA amiloidosis. There is no specific laboratory or radiological finding. Colchicum which is used in the treatment, decreases frequency of attacks and prevents from development of amiloidosis. Result: Aproximately 1/4 patients are resistive to colchicum treatment. There are some studies which shows adding interferon a, selective seratonine reuptake inhibitors and biological agents to the treatment are effective in these patients.
Abstract (Original Language): 
Amaç: Ailesel Akdeniz Ateşi, günümüzde bilinen herediter periyodik ateş sendromları arasında en yaygın ve en iyi tanınmış olanıdır. Biz bu derlemede, yurdumuzda sıkça görülen bu hastalığı ve yeni tedavi metotlarını özetledik. Ana bulgular: Ailesel Akdeniz ateşi literatürde ilk defa 1908 yılında bir Yahudi kızında tanımlanmıştır. Çoğunlukla Ermenilerde, Sefarad Yahudilerinde, Araplarda, Anadolu Türklerinde görülür. Hastalığın patogenezi tam olarak aydınlatılamamıştır. Tipik özelliği karın ağrısı, göğüs ağrısı veya eklem ağrısının eşlik ettiği akut ateş ataklarıdır. En önemli komplikasyonu ise AA tipi amiloidozdur. Spesifik bir laboratuvar veya radyolojik bulgusu yoktur. Tedavide en çok kullanılan kolşisin, akut atak sıklığını azalttığı gibi amiloidoz gelişimini önlemede de etkindir. Sonuç: Hastaların yaklaşık 1/4'ü kolşisin tedavisine dirençlidir. Bu hastalarda tedaviye eklenen interferon a, selektif ser-atonin reuptake inhibitörleri veya biyolojik ajanların etkili olduğunu gösteren çalışmalar vardır.
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