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- W2913376420 endingPage "e76" @default.
- W2913376420 startingPage "e61" @default.
- W2913376420 abstract "Familial Mediterranean Fever (FMF) is the oldest and the most frequent of all described hereditary periodic fever syndromes. The populations originating from Mediterranean basin carry the highest risk for FMF however it is being increasingly recognized in many parts of the world. It is an autoinflammatory disease with an autosomal recessive transmission. In the majority of the patients it is related with mutations in the MEFV gene that encodes a protein named pyrin. This protein has been shown to act as a regulator of inflammation mediated by IL-1β, which plays a major role in the pathogenesis of FMF. Approximately one-third of the patients have either a single or no mutation which raise questions about its mode of inheritance. FMF is a clinical diagnosis and characterized by self-limited bouts of fever and serositis. The main long-term complication of the disease is AA amyloidosis. The mainstay of treatment is life-long colchicine given daily to prevent the recurrence of febrile attacks and the development of amyloidosis. Patients with insufficient response to colchicine may be treated with anti IL-1 agents." @default.
- W2913376420 created "2019-02-21" @default.
- W2913376420 creator A5039434152 @default.
- W2913376420 creator A5075666115 @default.
- W2913376420 date "2019-02-01" @default.
- W2913376420 modified "2023-10-14" @default.
- W2913376420 title "Familial Mediterranean Fever" @default.
- W2913376420 cites W1525937482 @default.
- W2913376420 cites W1525971732 @default.
- W2913376420 cites W1598737571 @default.
- W2913376420 cites W1669620694 @default.
- W2913376420 cites W1754287932 @default.
- W2913376420 cites W1929086854 @default.
- W2913376420 cites W1964704186 @default.
- W2913376420 cites W1965301450 @default.
- W2913376420 cites W1967313258 @default.
- W2913376420 cites W1967629686 @default.
- W2913376420 cites W1968496780 @default.
- W2913376420 cites W1972254172 @default.
- W2913376420 cites W1973875123 @default.
- W2913376420 cites W1978299566 @default.
- W2913376420 cites W1980442164 @default.
- W2913376420 cites W1982460028 @default.
- W2913376420 cites W1986933064 @default.
- W2913376420 cites W1988455358 @default.
- W2913376420 cites W1992204034 @default.
- W2913376420 cites W1995521415 @default.
- W2913376420 cites W1997187737 @default.
- W2913376420 cites W1997702653 @default.
- W2913376420 cites W2000017388 @default.
- W2913376420 cites W2002511600 @default.
- W2913376420 cites W2004460528 @default.
- W2913376420 cites W2006781264 @default.
- W2913376420 cites W2008867503 @default.
- W2913376420 cites W2012145456 @default.
- W2913376420 cites W2012526145 @default.
- W2913376420 cites W2014359667 @default.
- W2913376420 cites W2015202335 @default.
- W2913376420 cites W2015206858 @default.
- W2913376420 cites W2017480987 @default.
- W2913376420 cites W2019555509 @default.
- W2913376420 cites W2021034392 @default.
- W2913376420 cites W2023850046 @default.
- W2913376420 cites W2025281034 @default.
- W2913376420 cites W2026688768 @default.
- W2913376420 cites W2028447626 @default.
- W2913376420 cites W2029446881 @default.
- W2913376420 cites W2032006559 @default.
- W2913376420 cites W2032140602 @default.
- W2913376420 cites W2033698154 @default.
- W2913376420 cites W2045759307 @default.
- W2913376420 cites W2048246503 @default.
- W2913376420 cites W2051713346 @default.
- W2913376420 cites W2052886925 @default.
- W2913376420 cites W2054134299 @default.
- W2913376420 cites W2056117282 @default.
- W2913376420 cites W2062073449 @default.
- W2913376420 cites W2064077696 @default.
- W2913376420 cites W2064571598 @default.
- W2913376420 cites W2065623147 @default.
- W2913376420 cites W2065845748 @default.
- W2913376420 cites W2067325139 @default.
- W2913376420 cites W2068836068 @default.
- W2913376420 cites W2072148754 @default.
- W2913376420 cites W2074580772 @default.
- W2913376420 cites W2076352044 @default.
- W2913376420 cites W2080667972 @default.
- W2913376420 cites W2084129561 @default.
- W2913376420 cites W2087427136 @default.
- W2913376420 cites W2092168339 @default.
- W2913376420 cites W2095834327 @default.
- W2913376420 cites W2108373994 @default.
- W2913376420 cites W2111478618 @default.
- W2913376420 cites W2111571297 @default.
- W2913376420 cites W2112413460 @default.
- W2913376420 cites W2112875364 @default.
- W2913376420 cites W2119760857 @default.
- W2913376420 cites W2120729303 @default.
- W2913376420 cites W2122605756 @default.
- W2913376420 cites W2130580918 @default.
- W2913376420 cites W2135395364 @default.
- W2913376420 cites W2136071868 @default.
- W2913376420 cites W2136478009 @default.
- W2913376420 cites W2137505775 @default.
- W2913376420 cites W2140276493 @default.
- W2913376420 cites W2141257085 @default.
- W2913376420 cites W2141815856 @default.
- W2913376420 cites W2142685289 @default.
- W2913376420 cites W2146730043 @default.
- W2913376420 cites W2156410123 @default.
- W2913376420 cites W2156691539 @default.
- W2913376420 cites W2156994068 @default.
- W2913376420 cites W2158142197 @default.
- W2913376420 cites W2162647701 @default.
- W2913376420 cites W2166034546 @default.
- W2913376420 cites W2166304177 @default.
- W2913376420 cites W2167353300 @default.
- W2913376420 cites W2171015472 @default.