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- W2002581810 abstract "Hereditary periodic fever syndromes are defined as recurrent attacks of generalized inflammation for which no infectious or auto-immune cause can be identified. Minimal clinical variations, a unique biochemical-specific abnormality and the mode of genetic inheritance distinguish the four main diseases: familial Mediterranean fever, hyper-immunoglobulinemia D, TNF-receptor-associated periodic syndrome (TRAPS) and Muckle Wells syndrome. It presents with prolonged attacks of fever and severe localized inflammation. TRAPS is caused by dominantly inherited mutations in the gene encoding the first TNF receptor, which result in decreased serum levels of soluble TNF-receptor leading to inflammation due to unopposed TNF-alpha action. Corticosteroid treatment is not completely effective in most TRAPS patients. Preliminary experiences with recombinant TNF-receptor analogues in the treatment appear be promising." @default.
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- W2002581810 date "2003-01-01" @default.
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- W2002581810 title "Le TNF-receptor associated periodic syndrome (TRAPS) : aspects cliniques et physiopathologiques d'une maladie familiale rare" @default.
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- W2002581810 doi "https://doi.org/10.1016/s0929-693x(03)00222-7" @default.
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