LEADER 00812cam0 22002533 450 001 SOB0000003915 005 20200924072349.0 010 $a8400072790 100 $a20010319d1992 |||||ita|0103 ba 101 $aspa 102 $aSP 200 1 $aDali residente$fRafael Santos Torroela 210 $aMadrid$cPublicaciones de la Residencia de Estudiantes$d1992 215 $a250 p.$cill.$d24 cm. 700 1$aSantos_Torroella$b, Rafael$3AF00010127$4070$0780244 801 0$aIT$bUNISOB$c20200924$gRICA 850 $aUNISOB 852 $aUNISOB$j700$m89883 912 $aSOB0000003915 940 $aM 102 Monografia moderna SBN 941 $aM 957 $a700$b002318$gSI$d89883$rACQUISTO$1Spinosa$2UNISOB$3UNISOB$420191007122533.0$520191007122542.0$6Spinosa 996 $aDali residente$91678918 997 $aUNISOB LEADER 04072oam 22004815 450 001 9910369948603321 005 20240215181657.0 010 $a3-030-19055-2 024 7 $a10.1007/978-3-030-19055-2 035 $a(CKB)4100000009678361 035 $a(MiAaPQ)EBC5971218 035 $a(DE-He213)978-3-030-19055-2 035 $a(PPN)248603108 035 $a(EXLCZ)994100000009678361 100 $a20191029d2020 uy 0 101 0 $aeng 135 $aurcnu|||||||| 181 $ctxt$2rdacontent 182 $cc$2rdamedia 183 $acr$2rdacarrier 200 10$aPeriodic and non-periodic fevers /$fedited by Rolando Cimaz 205 $a1st ed. 2020. 210 1$aCham :$cSpringer International Publishing :$cImprint: Springer,$d2020. 215 $a1 online resource (287 pages) $cillustrations (some color) 225 1 $aRare Diseases of the Immune System,$x2282-6505 311 0 $a3-030-19054-4 320 $aIncludes bibliographical references and index. 327 $aPreface -- I From Pathogenesis to Classification -- 1 Historical notes on Autoinflammatory disorders -- 2 Pathogenesis:Genetic basis, Innate Immunity, Inflammosomes, Cytokines -- 3 Classification and nomenclature of Periodic and Non Periodic fevers -- II Specific diseases -- 4 Mevalonate Kinase Deficiency (MKD) -- 5 Tumor necrosis factor receptors associated syndrome (TRAPS) -- 6 Cryopyrin-Associated Periodic Syndromes (CAPS) -- 7 Pyogenic Arthritis, Pyoderma gangrenosum and Acne Syndrome (PAPA) -- 8 Adenosine deaminase syndrome 2 (ADA2) -- 9 Interferonopathies -- 10 Inflammatory bone disease -- 11 Amyloidosis -- 12 Dermatologic autoinflammatory diseases -- 13 Autoinflammatory diseases with immunodeficiency -- 14 Periodic Fever, Aphthous Stomatitis, Pharyngitis, Adenitis Syndrome (PFAPA) -- 15 Proteasome. related disorders -- 16 Chronic Non-bacterial osteomyelitis -- 17 Systemic JIA and adult onset Still disease -- 18 Behcet disease -- 19 Recurrent idiopathic pericarditis -- 20 Schnitzler syndrome -- Subject Index. 330 $aThis book, written by very well-known opinion leaders in the field, covers all aspects of periodic and non ?periodic fevers, and related disorders. The expression refers to several different auto-inflammatory diseases, showing similar symptoms?the primary symptom being a recurrent fever for an infectious cause cannot be found. The opening chapters give some historical hints, explain the genetic basis of the disease and provide insights into the pathogenesis derived from recent experimental studies and guides the reader through classification and nomenclature. A large part of the book is then devoted to a detailed description of the specific related diseases and their clinical presentations, the disease course, and potential complications in both pediatric and adult patients. The advice regarding treatment is based on the best currently available evidence in this constantly evolving area. The book is part of Springer?s series Rare Diseases of the Immune System, which presents recently acquired knowledge on pathogenesis, diagnosis, and therapy with the aim of promoting a more holistic approach to these conditions. Autoinflammatory diseases are hereditary disorders that are caused by single-gene defects in innate immune regulatory pathways and are characterized by a clinical and biological inflammatory syndrome in which there is limited, if any, evidence of autoimmunity. Periodic and Non-Periodic Fevers will be an invaluable source of up-to-date information for all practitioners involved in the care of patients with these disease. 410 0$aRare Diseases of the Immune System,$x2282-6505 606 $aFever 606 $aPeriodic diseases 615 0$aFever. 615 0$aPeriodic diseases. 676 $a616.047 702 $aCimaz$b Rolando$4edt$4http://id.loc.gov/vocabulary/relators/edt 801 0$bMiAaPQ 801 1$bMiAaPQ 801 2$bMiAaPQ 906 $aBOOK 912 $a9910369948603321 996 $aPeriodic and Non-Periodic Fevers$92291679 997 $aUNINA