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020 _a9783319917856
024 7 _a10.1007/978-3-319-91785-6
_2doi
040 _bspa
_cES-MaUEC
_dES-MaUEC
050 4 _aRC606
_b.H86 2019 EB
245 0 0 _aHumoral Primary Immunodeficiencies
_cedited by Mario Milco D'Elios, Marta Rizzi
264 1 _aCham
_bSpringer International Publishing :
_bImprint: Springer
_c2019
300 _a1 recurso en línea (X, 387 páginas 34 ilustraciones a color)
336 _2rdacontent
_aTexto (visual)
_btxt
337 _2rdamedia
_aelectrónico
_bc
338 _2rdacarrier
_arecurso electrónico
_bcr
347 _atext file
_bPDF
_2rda
490 1 _aRare Diseases of the Immune System,
_x2282-6505
490 0 _aMedicine (Springer-11650)
505 0 _aPreface -- 1. B cells and the B-cell immunological synapse.-2 Mucosal B cells -- 3 CVID. – 4 LOCID. – 5 Genetics of CVID. – 6 ICOS deficiency. – 7 CD19 deficiency -- 8 CD20 deficiency. – 9 CD21 deficiency. – 10 CD81 deficiency. - 11 TACI deficiency, . – 12 LRBA deficiency. – 13 BAFF receptor deficiency. – 14 TWEAK defects. - 15 NFKB2 deficiency. – 16 WHIM syndrome. – 17 Novel humoral PID genes. – 18 Class Switch Recombination defects, -- 19 Selective IgA deficiency -- 20 IgA with IgG subclass deficiency. - 21 Isolated IgG subclass deficiency. – 22 Thymoma with immunodeficiency. – 23 PRKC delta and PI3K-delta associated diseases. – 24 Differential diagnostic in hypogammaglobulinemia. – 25 Management of Humoral primary immunodeficiencies in pediatrics -- 26 Management of Humoral primary immunodeficiencies in adults -- 27 Vaccines in humoral PID -- 28 Humoral PID and neoplasias -- 29 Humoral PID and autoimmunity. – 30 Lung involvement -- 31 Gut involvement -- Subject Index.
520 3 _aThis book presents detailed state of the art knowledge on the humoral primary immunodeficiencies (PIDs), i.e., disorders arising from impaired antibody production due to defects intrinsic to B cells or defective interaction between B and T cells. There is extensive coverage of both basic science discoveries and the latest clinical advances in the field. The book is structured in accordance with the most recent classification of PIDs and also covers updates on the B cell immunological synapse. Readers will find comprehensive, in-depth descriptions of novel humoral PID genes and related clinical applications, mucosal B cells, and the various clinical phenotypes of humoral PIDs. Aspects such as differential diagnosis, clinical management in children and adults, and the role of vaccines are also addressed. The authors are all recognized experts from Europe, Australia, and the United States. Humoral Primary Immunodeficiencies will be of high value for immunologists, pediatricians, rheumatologists, oncologists, internists, and infectious disease specialists and will also be informative for MD and PhD students.
988 _aPrimersemestre_2019_Medicine
650 7 _2embne
_9667061
_aInmunodeficiencia
650 7 _2embne
_9160812
_aEnfermedades raras
700 1 _aD'Elios, Mario Milco.
_eeditor literario
_4edt
_4http://id.loc.gov/vocabulary/relators/edt
700 1 _aRizzi, Marta.
_eeditor literario
_4edt
_4http://id.loc.gov/vocabulary/relators/edt
710 2 _aSpringerLink (Online service)
_9106937
776 0 8 _iPrinted edition:
_z9783319917849
776 0 8 _iPrinted edition:
_z9783319917863
830 0 _aRare Diseases of the Immune System,
_x2282-6505
856 4 0 _uhttps://go.openathens.net/redirector/universidadeuropea.es?url=https://doi.org/10.1007/978-3-319-91785-6
_zAcceso a este recurso digital (usuarios Universidad Europea de Valencia)
942 _2lcc
_cLE
998 _db
_feng
_ggw
_h0
_el
_zSI