LEADER 03857nam 22007095 450 001 9910483482603321 005 20251222132543.0 010 $a3-030-70107-7 024 7 $a10.1007/978-3-030-70107-9 035 $a(CKB)5590000000486813 035 $a(DE-He213)978-3-030-70107-9 035 $a(MiAaPQ)EBC6640491 035 $a(Au-PeEL)EBL6640491 035 $a(OCoLC)1256541725 035 $a(PPN)258062665 035 $a(EXLCZ)995590000000486813 100 $a20210610d2021 u| 0 101 0 $aeng 135 $aurnn#008mamaa 181 $ctxt$2rdacontent 182 $cc$2rdamedia 183 $acr$2rdacarrier 200 10$aCellular Primary Immunodeficiencies /$fedited by Mario Milco D'Elios, Cosima Tatiana Baldari, Francesco Annunziato 205 $a1st ed. 2021. 210 1$aCham :$cSpringer International Publishing :$cImprint: Springer,$d2021. 215 $a1 online resource (XIV, 513 p. 41 illus., 38 illus. in color.) 225 1 $aRare Diseases of the Immune System,$x2283-6403 311 08$a3-030-70106-9 327 $a1. Primary Immunodeficiencies -- 2. Genetics of Cellular Immunodeficiencies -- 3. Phenocopies of Primary Immuno Deficiencies -- 4. Dysfunctional immune synapses in T cell immunodeficiencies -- 5. Mucosal immunity in primary immunodeficiencies -- 6. Combined Immuno Deficiencies -- 7. Congenital defects of phagocytes -- 8. Defects in intrinsic and innate immunity -- 9. Diseases of immune dysregulation -- 10. IPEX syndrome and IPEX-related disorders -- 11. Autoinflammatory disorders -- 12. Lung in Cellular Immunodeficiencies -- 13. Gut involvement in Cellular Immunodeficencies -- 14. Malignancies in Cellular Immunodeficencies -- 15. Autoimmunity in Cellular Immunodeficencies -- 16. Differential diagnostic in Cellular Immunodeficiencies -- 17. Management of Cellular Immunodeficiencies -- 18. Vaccines in Cellular Immunodeficiencies -- 19. Gene therapy in Cellular Immunodeficiencies. 330 $aThis volume of the series Rare Diseases of the Immune System presents detailed state of the art knowledge on the cellular primary immunodeficiencies; it includes extensive coverage of both basic science discoveries and the latest clinical advances in the field. The book is structured in accordance with the most recent classification of PIDs and also covers updates on the T cell immunological synapse. Readers will find comprehensive, in-depth descriptions of novel cellular PID genes and related clinical applications, mucosal T cells, and the various clinical phenotypes of cellular PIDs. Cellular Primary Immunodeficiencies will be of high value for immunologists, pediatricians, rheumatologists, oncologists, internists, and infectious disease specialists and will also be informative for MD, Master and PhD students. 410 0$aRare Diseases of the Immune System,$x2283-6403 606 $aRheumatology 606 $aImmunology 606 $aMedical genetics 606 $aRheumatology 606 $aImmunology 606 $aClinical Genetics 606 $aImmunodeficiència$2thub 606 $aPatologia cel·lular$2thub 606 $aSistema immunitari$2thub 608 $aLlibres electrònics$2thub 615 0$aRheumatology. 615 0$aImmunology. 615 0$aMedical genetics. 615 14$aRheumatology. 615 24$aImmunology. 615 24$aClinical Genetics. 615 7$aImmunodeficiència 615 7$aPatologia cel·lular 615 7$aSistema immunitari 676 $a616.0473 702 $aD'Elios$b Mario Milco 702 $aBaldari$b Cosima Tatiana 702 $aAnnunziato$b Franceso 702 $aLippi$b Donatella 801 0$bMiAaPQ 801 1$bMiAaPQ 801 2$bMiAaPQ 906 $aBOOK 912 $a9910483482603321 996 $aCellular Primary Immunodeficiencies$92208975 997 $aUNINA