Familial Mediterranean Fever in Türkiye

Abstract

Ocak 1958–Haziran 1969 arasında Ankara Üniversitesi II. İç Hastalıkları Kliniğinde 77 Türk hastada–periyodik ateş, serozit atağı (peritonit, plörit), eklem ağrıları ve karın ağrısı gibi–ailevi Akdeniz humması (AAH) bulguları saptandı. Başlangıç yaşı 1–48 (ortalama 28) arasında olup vakaların %75’i 20 yaşından önce atağa başladı. Nöbetler 1–6 gün sürdü, lökositoz ve yüksek sedimentasyon oranı yaygındı, %62 olguda proteinüri görüldü; 48 proteinürik hastanın 45’ine yapılan böbrek biyopsisinde %57 amiloidozis doğrulandı. Literatürdeki 49 Türk vakanın eklenmesiyle toplam AAH’lı Türk sayısı 126’ya ulaştı. Bulgular, AAH’nın Türk popülasyonunda sık rastlanan genetik bir serozit sendromu olduğunu ve amiloid böbrek tutulumu başlıca komplikasyonunu oluşturduğunu göstermektedir.

Türkiye'de Ailevi Akdeniz Ateşi

Abstract

From January 1958 through June 1969, 77 Turkish patients with clinical features of familial Mediterranean fever (FMF)—paroxysmal fever attacks, serositis (peritonitis, pleuritis), arthralgias, and abdominal pain—were identified at Ankara University’s Second Department of Internal Medicine. Ages at onset ranged from 1 to 48 years (mean 28), with 75% presenting before age 20. Recurrent attacks lasted 1–6 days at irregular intervals. Laboratory findings included leukocytosis, elevated sedimentation rate, and proteinuria in 62% of cases; renal biopsy in 45 of 48 proteinuric patients confirmed amyloidosis in 57%. A review of the Turkish and international literature added 49 previously reported Turkish cases, bringing the total to 126. These data demonstrate that FMF is a significant hereditary serositis syndrome among Turks, with amyloid nephropathy as its most serious complication.

Keywords

• familial Mediterranean fever
• FMF
• amyloidosis
• hereditary serositis

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