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TÜRK TIBBINDA AİLEVİ AKDENİZ ATEŞÎ HASTALIĞI VE AMÎLOÎDOZ

FAMILIAL MEDITERRANEAN FEVER AND AMILOIDOSIS IN TURKISH MEDICINE

Journal Name:

  • Türk Nefroloji, Diyaliz ve Transplantasyon Dergisi

Publication Year:

  • 2003

Key Words:

Keywords (Original Language):

Author NameUniversity of AuthorFaculty of Author
Abstract (2. Language): 
In this report, by the guidance of Turkish and international literature, the features of FMF disease in Turkish patients and the advances in related with thi disease Turkish Medicine were presented. FMF is an autosomal recessesive hereditary disease which affects people of mediterranean origins (Turks, Armenians, Araps, Sephardic Jews). The disease is characterized by recurrent, self-limited fever, abdominal pain, pleuritis, arthritis and erysipelas-like skin lesion. FMF is a disease of childhood and %90 of patients begin to suffer before 20 years of age. The clinical features are similiar in all affected ethnic groups. But it was emphasised by several reports that course of the disease is more severe and the incidence of amyloidosis is much higher in Turkish patients compared to other ethnic groups. The diagnosis of FMF is based on clinical manifestations and following up patients. FMF suspectible patients should be evaluated both in attacks and attack-free period for accurate diagnosis. There is no spesific laboratory tests for FMF. During attacks, acute phase reactants such as C-reactive protein, fibrinogen, ceruloplazmin, serum amyloid A are elevated. All these tests are usually returned gradually to normal values after attacks. The most important complication of FMF is amyloidosis, usually affecting the kidneys, resulting in renal insufficiency Today, colchicine treatment decreases the occurance of FMF attacks and prevents development of amyloidosis Previovsly as the knowledge and experiences about the disease increases, patients are more regularly observed and receivel colchicine treatment. Consequently amyloidosis has been decreased in Turkey as well as in the world
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Abstract (Original Language): 
Bu yazıda, Türk ve dünya literatürlerinin ışığında Türklerde FMF hastalığının özellikleri ve Türk tıbbındaki hastalıkla ilgili gelişmeler sunulmuştur. FMF; Akdeniz kökenli toplumlarda sık görülen, tekrarlayan ve kendi kendini sınırlayan ateş ile birlikte karın ağrısı, plevrit, artrit ve erizipel benzeri deri lezyonu ile karakterize otozomal resesif geçişli bir hastalıktır. FMF, esas olarak çocukluk çağı hastalığıdır ve hastaların %90'ında şikayetler 20 yaşından önce başlar. Hastalığın klinik özelliklen farklı etnik gruplarda birbirine benzemektedir. Ancak Türklerde hastalığın daha ağır seyrettiği ve amiloid gelişme riskinin daha yüksek olduğu çeşitli yayınlarda vurgulanmıştır. Ailevi Akdeniz Ateşi tanısı klinik bulgular ve hasta izlemiyle konur. Tanının konulabilmesi için hastanın mutlaka atak döneminde ve ataksız dönemde değerlendirilmesi gerekir. Ailevi Akdeniz ateşi tanısı için spesifik bir laboratuvar testi yoktur. Akut inflamasyonla giden olaylarda arttığı bilinen akut faz proteinleri (CRP, fibrinojen, seruloplazmin, serum amiloid A proteini vb) atakta artar ve atak sonrasında normale döner. FMF hastalarını tehdit eden en önemli komplikasyon böbrek yetmezliğine yol açabilen amiloidozistir. Günümüzde FMF hastalarına atakların azalmasını sağlayan ve daha da önemlisi tüm hastalarda amiloid gelişimini önleyen kolşisin tedavisi uygulanmaktadır. Son yıllarda hastalıkla ilgili bilgi ve deneyimlerin artması hastaların daha düzenli izlenmesi ve dünyada olduğu kadar Türkiye'de de amiloid vakalarının azalması sonucunu doğurmuştur.
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