Humoral Primary Immunodeficiencies / edited by Mario Milco D'Elios, Marta Rizzi.

Format:

Book

Contributor:

D'Elios, Mario Milco, editor.

Rizzi, Marta, editor.

SpringerLink (Online service)

Series:

Medicine (Springer-11650)

Rare diseases of the immune system 2282-6505

Rare Diseases of the Immune System, 2282-6505

Language:

English

Subjects (All):

Rheumatology.

Immunology.

Infectious diseases.

Oncology.

Infectious Diseases.

Local Subjects:

Rheumatology.

Immunology.

Infectious Diseases.

Oncology.

Physical Description:

1 online resource (X, 387 pages) : 34 illustrations in color.

Edition:

First edition 2019.

Contained In:

Springer eBooks

Place of Publication:

Cham : Springer International Publishing : Imprint: Springer, 2019.

System Details:

text file PDF

Summary:

This book presents detailed state of the art knowledge on the humoral primary immunodeficiencies (PIDs), id est, 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:

Preface

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.

Other Format:

Printed edition:

ISBN:

978-3-319-91785-6

9783319917856

Access Restriction:

Restricted for use by site license.