LEADER 04461nam 22007215 450 001 9910300227703321 005 20250609110844.0 010 $a3-319-14615-7 024 7 $a10.1007/978-3-319-14615-7 035 $a(CKB)3710000000378055 035 $a(EBL)2095938 035 $a(SSID)ssj0001465502 035 $a(PQKBManifestationID)11817136 035 $a(PQKBTitleCode)TC0001465502 035 $a(PQKBWorkID)11471873 035 $a(PQKB)10031982 035 $a(DE-He213)978-3-319-14615-7 035 $a(MiAaPQ)EBC2095938 035 $a(PPN)184893941 035 $a(MiAaPQ)EBC3108644 035 $a(EXLCZ)993710000000378055 100 $a20150319d2015 u| 0 101 0 $aeng 135 $aur|n|---||||| 181 $ctxt 182 $cc 183 $acr 200 10$aFamilial Mediterranean Fever /$fedited by Marco Gattorno 205 $a1st ed. 2015. 210 1$aCham :$cSpringer International Publishing :$cImprint: Springer,$d2015. 215 $a1 online resource (166 p.) 225 1 $aRare Diseases of the Immune System,$x2282-6505 ;$v3 300 $aDescription based upon print version of record. 311 08$a3-319-14614-9 320 $aIncludes bibliographical references at the end of each chapters and index. 327 $aPreface -- 1 Genetics.- 2 Pathogenesis.- 3 Clinical Picture in Childhood -- 4 Clinical Picture in Adulthood and Unusual Clinical Features.- 5 FMF in Western Countries.- 6 Long Term Complications in FMF -- 7 Recent advances in quantitative assessment of FMF -- 8 How to Manage FMF Patients in Daily Practice -- 9 New Emerging Treatments.- Subject Index. 330 $aThis book, written by very well known opinion leaders in the field, covers all aspects of familial Mediterranean fever, the most common monogenic autoinflammatory disease. The opening chapters explain the genetic basis of the disease and provide insights into the pathogenesis derived from recent experimental studies. A large part of the book is then devoted to a detailed description of the typical and atypical clinical presentations, the disease course, and potential complications in both pediatric and adult patients. Guidance is provided on the measurement of disease severity and the management of patients in daily practice. The advice regarding treatment is based on the best currently available evidence and attention is also paid to important emerging treatments. The book is part of Springer?s series Rare Diseases of the Immune System, which presents recently acquired knowledge on pathogenesis, diagnosis, and therapy with the aim of promoting a more holistic approach to these conditions. Monogenic autoinflammatory diseases are hereditary disorders that are caused by single-gene defects in innate immune regulatory pathways and are characterized by a clinical and biological inflammatory syndrome in which there is limited, if any, evidence of autoimmunity. Familial Mediterranean fever itself is due to a mutation in the MEFV gene, which codes for the protein pyrin; it is characterized by periodic fever and episodes of painful inflammation in the abdomen, chest, and joints. Familial Mediterranean Fever will be an invaluable source of up-to-date information for all practitioners involved in the care of patients with the disease. 410 0$aRare Diseases of the Immune System,$x2282-6505 ;$v3 606 $aRheumatology 606 $aImmunology 606 $aInternal medicine 606 $aHuman genetics 606 $aRheumatology$3https://scigraph.springernature.com/ontologies/product-market-codes/H33170 606 $aImmunology$3https://scigraph.springernature.com/ontologies/product-market-codes/B14000 606 $aInternal Medicine$3https://scigraph.springernature.com/ontologies/product-market-codes/H33002 606 $aHuman Genetics$3https://scigraph.springernature.com/ontologies/product-market-codes/B12008 615 0$aRheumatology. 615 0$aImmunology. 615 0$aInternal medicine. 615 0$aHuman genetics. 615 14$aRheumatology. 615 24$aImmunology. 615 24$aInternal Medicine. 615 24$aHuman Genetics. 676 $a612.022 702 $aGattorno$b Marco$4edt$4http://id.loc.gov/vocabulary/relators/edt 801 0$bMiAaPQ 801 1$bMiAaPQ 801 2$bMiAaPQ 906 $aBOOK 912 $a9910300227703321 996 $aFamilial Mediterranean Fever$91762429 997 $aUNINA