By Marco Gattorno
This booklet, written through rather well identified opinion leaders within the box, covers all features of familial Mediterranean fever, the most typical monogenic autoinflammatory ailment. the hole chapters clarify the genetic foundation of the ailment and supply insights into the pathogenesis derived from fresh experimental reviews. a wide a part of the booklet is then dedicated to an in depth description of the common and peculiar medical displays, the illness path, and strength problems in either pediatric and grownup sufferers. assistance is supplied at the size of affliction severity and the administration of sufferers in day-by-day perform. the recommendation concerning therapy is predicated at the most sensible at the moment on hand facts and a focus is usually paid to big rising treatments.
The ebook is a part of Springer’s sequence Rare ailments of the Immune System, which offers lately received wisdom on pathogenesis, analysis, and remedy with the purpose of marketing a extra holistic method of those stipulations. Monogenic autoinflammatory illnesses are hereditary issues which are because of single-gene defects in innate immune regulatory pathways and are characterised by way of a scientific and organic inflammatory syndrome during which there's constrained, if any, facts of autoimmunity. Familial Mediterranean fever itself is because of a mutation within the MEFV gene, which codes for the protein pyrin; it truly is characterised via periodic fever and episodes of painful irritation within the stomach, chest, and joints. Familial Mediterranean Fever can be a useful resource of up to date details for all practitioners enthusiastic about the care of sufferers with the disease.
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Additional resources for Familial Mediterranean Fever
A survey of 470 cases and review of the literature. Am J Med. 1967;43(2):227–53. 69. Staub E, Dahl E, et al. The DAPIN family: a novel domain links apoptotic and interferon response proteins. Trends Biochem Sci. 2001;26(2):83–5. 70. Stehlik C, Fiorentino L, et al. The PAAD/PYRIN-family protein ASC is a dual regulator of a conserved step in nuclear factor kappaB activation pathways. J Exp Med. 2002;196(12):1605–15. 71. Touitou I. The spectrum of Familial Mediterranean Fever (FMF) mutations. Eur J Hum Genet.
Ozcakar ZB, et al. MEFV mutations modify the clinical presentation of Henoch-Schonlein purpura. J Rheumatol. 2008;35:2427–9. 47. Ozen S, et al. Polyarteritis nodosa in patients with Familial Mediterranean Fever (FMF): a concomitant disease or a feature of FMF? Semin Arthritis Rheum. 2001;30:281–7. 19958. 48. Hull KM, Shoham N, Chae JJ, et al. The expanding spectrum of systemic autoinflammatory disorders and their rheumatic manifestations. Curr Opin Rheumatol. 2003;15:61–9. 49. Ben-Chetrit E, Levy M.
Familial Mediterranean fever. A survey of 470 cases and review of the literature. Am J Med. 1967;43(2):227–53. 69. Staub E, Dahl E, et al. The DAPIN family: a novel domain links apoptotic and interferon response proteins. Trends Biochem Sci. 2001;26(2):83–5. 70. Stehlik C, Fiorentino L, et al. The PAAD/PYRIN-family protein ASC is a dual regulator of a conserved step in nuclear factor kappaB activation pathways. J Exp Med. 2002;196(12):1605–15. 71. Touitou I. The spectrum of Familial Mediterranean Fever (FMF) mutations.