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Table of Contents:

Preface
Introduction: Primary Immunodeficiency Diseases - Report of a WHO Scientific Group 1
I Primary Specific Immunodeficiencies
X Chromosome Linked Immunodeficiency 31
Immunodeficiency with Hyper-IgM (HIM) 45
IgG Subclass Deficiency 61
IgA Deficiency 77
Gene Deletions in the Human Immunoglobulin Heavy Chain Constant Region Locus: Molecular and Immunological Analysis 99
Cellular Abnormalities in Common Variable Immunodeficiency 111
Severe Combined Immunodeficiencies 127
Combined Immunodeficiency with Defective Expression in MHC Class II Genes 141
Congenital T-Cell Receptor Immunodeficiencies in Man 155
The DiGeorge Anomaly 167
Adenosine Deaminase Deficiency 177
Purine Nucleoside Phosphorylase Deficiency 197
Sialophorin (CD43) and the Wiskott-Aldrich Syndrome 225
Genetic Aspects of Ataxia-Telangiectasia 243
II Complement Deficiencies
Inherited Deficiency of the Fourth Component of Human Complement 253
Hereditary and Acquired Deficiencies of C1 Inhibitor 267
Deficiency of the First Component of Human Complement 283
Inherited Deficiencies of the Terminal Components of Human Complement 295
Deficiencies of Human C3 Complement Receptors Type 1 (CR1, CD35) and Type 2 (CR2, CD21) 313
III Leukocyte Defects
Updating on the Leukocyte Adhesion Deficiency 333
Molecular Genetics of Chronic Granulomatous Disease 335
Molecular Basis of the Autosomal Recessive Forms of Chronic Granulomatous Disease 347
IV HIV Disease
The Role of the CD4 Molecule in the Immune System and HIV Infection 365
Immunological Abnormalities in the Natural History of HIV Infection: Mechanisms and Clinical Relevance 385
Cytotoxic T Lymphocytes in HIV-induced Disease: Implications for Therapy and Vaccination 401
The Programmed Cell Death Theory of AIDS Pathogenesis: Implications, Testable Predictions, and Confrontation with Experimental Findings 419
Human Immunodeficiency Virus Type 1 (HIV-1) Infection of the Nervous System: A Review 427
HIV-related Thrombocytopenia 457
Pediatric HIV Infection: A Review 465
Perinatal HIV Transmission: Facts and Controversies 509
Early Therapeutic Intervention in HIV Infection: Stepping Stones to Success 527
Drug Resistance in Isolates of HIV 537
AIDS Vaccines: Concepts and First Trials 545
V Secondary Immunodeficiencies Other than AIDS
Immunodeficiencies in Parasitic Diseases 559
Hyper Immunoglobulin E Syndrome 571
X-linked Lymphoproliferative Syndrome 585
VI Animal Models
B and T Cell Leakiness in the Scid Mouse Mutant 601
The Xlr (X-linked Lymphocyte Regulated) Gene Family (A candidate locus for an X-linked primary immune deficiency) 613
Domestic Animal Models of Severe Combined Immunodeficiency: Canine X-linked Severe Combined Immunodeficiency and Severe Combined Immunodeficiency in Horses 629
Nonhuman Primate Models for HIV Vaccine Development 649
VII Therapy
Intravenous Immunoglobulin: A Review 659
The Use of HLA-Non-identical T-Cell-depleted Marrow Transplants for Correction of Severe Combined Immunodeficiency Disease 689
Human Graft-versus-Host Disease 717
Gene Therapy for Primary Immunodeficiency Disease 727
Index 743