New Concepts in Immunodeficiency Diseases / Edition 1

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Rapid progress has been made in the understanding of the basic concepts of the immune response at biochemical and molecular levels. This text reviews the development of innovative techniques for diagnosing disorders at a very early stage and for detecting their carrier states and discusses novel therapeutic approaches such as gene therapy and the use of biological response modifiers.
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Editorial Reviews

During the past decade, rapid progress has been made in the understanding of the basic concepts of immune response at the biochemical and molecular levels. This new information has been instrumental in understanding the defects of many primary immunodeficiency disorders at the molecular level. As a result, innovative techniques are now available to diagnose them at a very early stage and to detect their carrier states. Novel therapeutic approaches, including gene therapy and use of biological response modifiers, have emerged. This volume reviews the developing concepts in primary immunodeficiency disorders and is divided into three major sections: basic mechanisms in immune response; genetic and molecular basis of primary immunodeficiency disorders; and advances in therapy. Annotation c. Book News, Inc., Portland, OR (
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Product Details

  • ISBN-13: 9780471938804
  • Publisher: Wiley, John & Sons, Incorporated
  • Publication date: 1/28/1993
  • Edition number: 1
  • Pages: 518
  • Product dimensions: 6.23 (w) x 9.29 (h) x 1.31 (d)

Table of Contents

Pt. A Basic Mechanisms
1 Development of the Human Antibody Repertoire 3
2 Pathways of Human B Cell Growth and Differentiation 27
3 Substituted Guanine Ribonucleosides as B Cell Stimulators: Their Potential Clinical Applications 45
4 Development of the Human T Cell Repertoire 57
5 Signal Transduction in T Cell Activation and Tolerance 85
6 Adhesion Molecules which Guide Neutrophil-Endothelial Cell Interactions at Sites of Inflammation 131
Pt. B Immunodeficiency Diseases
7 X-linked Severe Combined Immunodeficiency 159
8 Combined Immunodeficiency with Defective Expression in MHC Class II Genes 177
9 Genetic Abnormalities in Leukocyte Adhesion Molecule Deficiency 191
10 Ataxia-Telangiectasia: Genetic Studies 203
11 Abnormalities of Signal Transduction and T Cell Immunodeficiency 231
12 The Wiskott-Aldrich Syndrome: An Immunodeficiency Associated with Defects of the CD43 Molecule 249
13 Complement Deficiency: C7, C8 and Properdin 269
14 Related Pathogenesis of Immunoglobulin A Deficiency and Common Variable Immunodeficiency 293
15 The Molecular Basis of Chronic Granulomatous Disease 311
Pt. C Therapy
16 Effects of Granulocyte Colony-stimulating Factor in Patients with Severe Chronic Neutropenia 355
17 Interferon [gamma] in Chronic Granulomatous Diseases of Childhood 371
18 Peripheral Stem Cell Transplantation 381
19 Interleukin 2 Deficiency in Primary Immunodeficiency: Exploration of Recombinant Interleukin 2 as a Potential In Vivo Treatment 391
20 The Role of Polyethylene Glycol-Adenosine Deaminase in the Evolution of Therapy for Adenosine Deaminase Deficiency 417
21 Gene Therapy for Immunodeficiency Disease 427
22 Intravenous Immunoglobulin: Current Concepts and Application 457
Index 477
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