Primary Immunodeficiency Disorders: A Historic and Scientific Perspective

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Amos Etzioni, Hans D. Ochs
Academic Press, Sep 13, 2014 - Medical - 376 pages

Primary Immunodeficiency Disorders: A Historic and Scientific Perspective provides a complete historical context that is crucial for students and researchers concerned with primary immunodeficiency. When researchers have a poor understanding of the way we arrived where we are in research, they can miss important points about a disease, or miss out on how to approach new diseases. This historical knowledge of research can assist greatly by showing how it was done in the past, demonstrating the successes and failures, so that it can be done better in the future.

This book provides an understanding of the process going from clinical problem to lab and back to the clinic, based on historical experiences. Its chapters proceed from the discovery of the T and B cell lineages through the first BMT for immunodeficiency disorder; lab investigation and gene therapy for PID; the discovery of the gene for AT and its function; understanding cytokine defects; and many other stops along the way.

  • Facilitates communication among physicians and other investigators concerned with immunological and inflammatory diseases
  • Summarizes for the first time all the known facts from 60 years of primary immunodeficiency research, and teaches how an important field in medicine was established
  • Provides stimulating discussions on developing new medical therapies Highlights the importance of studying humans to understand mechanisms of disease that affect humans
 

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Contents

From Serendipitous Observations to ScienceBased Specialty
1
Chapter 2 Discovery of the T and BCell Compartments
13
Chapter 3 Evolution of the Definition of Primary Immunodeficiencies
29
Chapter 4 From Immunodeficiency to Autoimmunity
41
Chapter 5 Immunological Tests from the Microscope to Whole Genome Analysis
51
Chapter 6 Primary Immunodeficiency in the Developing Countries
65
Chapter 7 Jeffrey Asked Us to Do Something Our Journey
77
Dates of description and gene discovery of major PIDDs
82
Chapter 15 Severe Combined Immunodeficiency as Diseases of Defective Cytokine Signaling
181
Chapter 16 The Hyper IgM Syndromes a Long List of Genes and Years of Discovery
197
Chapter 17 Unraveling the Complement System and its Mechanism of Action
217
A Serendipitous Discovery
229
Chapter 19 The Many Faces of the HyperIgE Syndrome
241
Chapter 20 ADA Deficiency The First Described Genetic Defect Causing PID
255
Chapter 21 The Leukocyte Adhesion Deficiency Story
267
Chapter 22 How Common Variable Immune Deficiency has Changed Over Six Decades
275

The AtaxiaTelangiectasia Gene and its Function
83
from a Fatal Hematologic Disorder to a Curable Immunodeficiency
97
Chapter 10 Neutropenia More Genetic Defects Than Ever Expected
111
from Xlinked Recessive to Autosomal Recessive Agammaglobulinemia
127
Chapter 12 The Discovery of the Familial Hemophagocytosis Syndromes
139
Chapter 13 Chronic Granulomatous Disease from a Fatal Disease to a Curable One
151
Chapter 14 Severe Combined Immunodeficiency from Discovery to Newborn Screening
171
Chapter 23 From Subcutaneous to Intravenous Immunoglobulin and Back
283
Chapter 24 History of Hematopoietic Stem Cell Transplantation
299
Chapter 25 Davids Story
313
Chapter 26 How Primary Immunodeficiencies Have Made Gene Therapy a Reality
327
Index
341
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