Familial Mediterranean fever (FMF) is classically an autosomalrecessive periodic inflammatory disease occurring in Mediterraneanand Middle Eastern populations. It is caused by mutations affectingboth alleles of MEFV, a gene that encodes pyrin (marenostrin), anuncharacterized neutrophil pro- tein. Occasional reports of autosomaldominant FMF have often been discounted, on the basis thatAsymptomatic FMF carriers are common in certain Populations, and giverise to pseudo-dominant Inheritance.
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