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020 _a9783319146157
024 7 _a10.1007/978-3-319-14615-7
_2doi
050 4 _aRB148
_b2015 EB
245 1 0 _aFamilial Mediterranean Fever
_cedited by Marco Gattorno.
260 _aCham, Switzerland
_bSpringer
_c2015
300 _a1 recurso en línea (VII, 162 páginas)
_b17 ilustraciones, 12 ilustraciones en color
336 _aTexto
_btxt
_2rdacontent
337 _aelectrónico
_bc
_2rdamedia
338 _arecurso electrónico
_bcr
_2rdacarrier
490 0 _aRare Diseases of the Immune System
_x2282-6505
_v3
505 0 _aPreface -- 1 Genetics.-Â{u2810}athogenesis.-Â{u3803}linical Picture in Childhood -- 4 Clinical Picture in Adulthood and Unusual Clinical Features.-Â{u5806}MF in Western Countries.-Â{u680C}ong 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.-Â{u3D62}ject Index.
520 3 _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 Springerâ€{u3833}eries 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 theÂ{uD146}FVÂ{u796E}e, 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.
710 2 _aSpringerLink (Online service)
_0Local
_9106996
942 _2lcc
_cLE
650 7 _aReumatología
_2embne
_9141883
650 7 _aGenética humana
_2embne
_9140562
650 7 _aInmunología
_2embne
_9138330
700 1 _aGattorno, Marco
_eeditor literario
_995138
_0Local
856 4 0 _uhttps://go.openathens.net/redirector/universidadeuropea.es?url=https://link.springer.com/book/10.1007/978-3-319-14615-7
_zAcceso a este recurso digital (usuarios Universidad Europea de Madrid)
907 _a.b12909403
_b10-10-17
_c10-02-16
988 _aSpringer_Medicine_2015
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_z06-04-17
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