Author:

Keywords:

Familiale Middellandse Zeekoorts

Abstract:

Familial Mediterranean fever: traditional concepts and recent advances-analysis of 22 patients in Belgium. Familial Mediterranean fever (FMF) is a hereditary autosomal recessive disease characterized by periodic attacks of fever and serositis. FMF is prevalent among populations of Midle Eastern and Mediterranean origin. In many countries where the native population is generally not affected, as in Belgium, people are less familiar with FMF. Still, a correct and early diagnosis remains of paramount importance, given the morbidity of FMF, its potentially lethal complications, and the availability of an effective therapy with colcichine. FMF is the prototype of the hereditary recurrent fevers (HRFs), a group af Mendelian diseases characterized by a seemingly unprovoked fever and inflammation. Starting with the identification of the FMF gene in 1997, many advances have been mad in the understanding of the genetics an the molecular biology of the hereditary fever syndromes. In this report, the clinical features, diagnosis, treatment, the pathogenesis and the molecular genetics of FMF are reviewed; the case histories of 22 patients with FMF followed in our centre are reported.