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010   $a3-319-91785-4
024 7 $a10.1007/978-3-319-91785-6
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035   $a(DE-He213)978-3-319-91785-6
035   $a(iGPub)SPNA0057491
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100   $a20181229d2019      u|         0
101 0 $aeng
135   $aurcnu||||||||
181   $ctxt$2rdacontent
182   $cc$2rdamedia
183   $acr$2rdacarrier
200 10$aHumoral Primary Immunodeficiencies /$fedited by Mario Milco D'Elios, Marta Rizzi
205   $a1st ed. 2019.
210  1$aCham :$cSpringer International Publishing :$cImprint: Springer,$d2019.
215   $a1 online resource (383 pages)
225 1 $aRare Diseases of the Immune System,$x2283-6403
311 08$a3-319-91784-6 
327   $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.
330   $aThis book presents detailed state of the art knowledge on the humoral primary immunodeficiencies (PIDs), i.e., disorders arising from impaired antibody production due to defects intrinsic to B cells or defective interaction between B and T cells. There is extensive coverage of both basic science discoveries and the latest clinical advances in the field. The book is structured in accordance with the most recent classification of PIDs and also covers updates on the B cell immunological synapse. Readers will find comprehensive, in-depth descriptions of novel humoral PID genes and related clinical applications, mucosal B cells, and the various clinical phenotypes of humoral PIDs. Aspects such as differential diagnosis, clinical management in children and adults, and the role of vaccines are also addressed. The authors are all recognized experts from Europe, Australia, and the United States. Humoral Primary Immunodeficiencies will be of high value for immunologists, pediatricians,rheumatologists, oncologists, internists, and infectious disease specialists and will also be informative for MD and PhD students.
410  0$aRare Diseases of the Immune System,$x2283-6403
606   $aRheumatology
606   $aImmunology
606   $aDiseases
606   $aOncology
606   $aRheumatology
606   $aImmunology
606   $aDiseases
606   $aOncology
615  0$aRheumatology.
615  0$aImmunology.
615  0$aDiseases.
615  0$aOncology.
615 14$aRheumatology.
615 24$aImmunology.
615 24$aDiseases.
615 24$aOncology.
676   $a616.979
702   $aD'Elios$b Mario Milco$4edt$4http://id.loc.gov/vocabulary/relators/edt
702   $aRizzi$b Marta$4edt$4http://id.loc.gov/vocabulary/relators/edt
906   $aBOOK
912   $a9910337498703321
996   $aHumoral Primary Immunodeficiencies$91736096
997   $aUNINA