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Humoral Primary Immunodeficiencies

https://libcat.nshealth.ca/en/permalink/provcat44219
Mario Milco D'Elios, Marta Rizzi, editors. --Cham: Springer , 2019.
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This 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. R…
Available Online
View e-Book
Other Authors
D'Elios, Mario Milco
Rizzi, Marta
Responsibility
Mario Milco D'Elios, Marta Rizzi, editors
Place of Publication
Cham
Publisher
Springer
Date of Publication
2019
Physical Description
1 online resource (x, 387 p.) : 34 illus. in color
Series
Rare Diseases of the Immune System
Series Title
Rare diseases of the immune system
ISBN
9783319917856
9783319917849 (Print ed.)
9783319917863 (Print ed.)
ISSN
2282-6505
Subjects (MeSH)
Immunity, Humoral - immunology
Immunologic Deficiency Syndromes - immunology
Immunologic Deficiency Syndromes - therapy
Specialty
Allergy and Immunology
Abstract
This 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.
Contents
1. The B-Side of the Immune Response -- 2. Mucosal B Cells -- 3. CVID -- 4. Late-Onset Combined Immunodeficiencies (LOCID) -- 5. Genetics of CVID -- 6. ICOS Deficiency -- 7. CD19 Deficiency due to Genetic Defects in the CD19 and CD81 Genes -- 8. Genetic CD21 Deficiency -- 9. TACI Deficiency -- 10. LRBA Deficiency -- 11. BAFF Receptor Deficiency -- 12. TWEAK Deficiency -- 13. NFKB2 Defects -- 14. The WHIM Syndrome -- 15. Class-Switch Recombination Defects -- 16. Selective IgA Deficiency -- 17. IgG Subclass and Anti-polysaccharide Antibody Deficiency -- 18. Good’s Syndrome (GS): Thymoma with Immunodeficiency -- 19. Differential Diagnosis in Hypogammaglobulinemia -- 20. Management of Humoral Primary Immunodeficiencies in Pediatrics -- 21. Management of Humoral Primary Immunodeficiencies in Adults -- 22. Vaccines in Humoral Primary Immunodeficiencies -- 23. Malignancy in Predominantly Antibody Deficiencies (PAD) -- 24. Humoral Primary Immunodeficiency and Autoimmune and Inflammatory Manifestations -- 25. Chest Complications in Patients with Primary Antibody Deficiency Syndromes (PADS) -- 26. Gastrointestinal Complications in Primary Immunoglobulin Deficiencies.
Format
e-Book
Location
Online
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