Contributors |
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xiii | |
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Genetically Determined Immunodeficiency Diseases: A Perspective |
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3 | (13) |
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Genetic Principles and Technologies in the Study of Immune Disorders |
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16 | (11) |
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Mammalian Hematopoietic Development and Function |
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27 | (12) |
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39 | (22) |
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Molecular Mechanisms Guiding B Cell Development |
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61 | (10) |
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Signal Transduction by T and B Lymphocyte Antigen Receptors |
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71 | (22) |
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Lymphoid Organ Development, Cell Trafficking, and Lymphocyte Responses |
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93 | (10) |
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103 | (20) |
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X-Linked Severe Combined Immunodeficiency |
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123 | (14) |
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Autosomal Recessive Severe Combined Immunodeficiency Due to Defects in Cytokine Signaling Pathways |
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137 | (16) |
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V(D)J Recombination Defects |
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153 | (16) |
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Immunodeficiency Due to Defects of Purine Metabolism |
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169 | (28) |
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Severe Combined Immunodeficiency Due to Mutations in the CD45 Gene |
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197 | (6) |
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Severe Combined Immunodeficiency Due to Defects in T Cell Receptor--Associated Protein Kinases |
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203 | (9) |
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Human Interleukin-2 Receptor α Deficiency |
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212 | (4) |
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216 | (11) |
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Molecular Basis of Major Histocompatibility Complex Class II Deficiency |
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227 | (15) |
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Barbara Lisowska-Grospierre |
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Peptide Transporter Defects in Human Leukocyte Antigen Class I Deficiency |
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242 | (9) |
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CD40, CD40 Ligand, and the Hyper-IgM Syndrome |
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251 | (18) |
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Autosomal Hyper-IgM Syndromes Caused by an Intrinsic B Cell Defect |
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269 | (10) |
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X-linked Agammaglobulinemia: A Disease of Btk Tyrosine Kinase |
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279 | (25) |
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Autosomal Recessive Agammaglobulinemia |
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304 | (9) |
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Genetic Approach to Common Variable Immunodeficiency and IgA Deficiency |
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313 | (13) |
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Autoimmune Lymphoproliferative Syndrome |
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326 | (16) |
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Autoimmune Polyendocrinopathy, Candidiasis, Ectodermal Dystrophy |
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342 | (13) |
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Immune Dysregulation, Polyendocrinopathy, Enteropathy, and X-Linked Inheritance |
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355 | (12) |
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367 | (23) |
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Inherited Disorders of the Interleukin-12/23-Interferon Gamma Axis |
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390 | (12) |
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402 | (25) |
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Chromosomal Instability Syndromes Other Than Ataxia-Telangiectasia |
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427 | (27) |
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454 | (16) |
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X-Linked Lymphoproliferative Disease Due to Defects of SH2DIA |
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470 | (15) |
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DiGeorge Syndrome: A Chromosome 22q11.2 Deletion Syndrome |
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485 | (11) |
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Hyper-IgE Recurrent Infection Syndromes |
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496 | (9) |
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Immunodeficiency with Centromere Instability and Facial Anomalies |
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505 | (8) |
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Immunodeficiencies with Associated Manifestations of Skin, Hair, Teeth, and Skeleton |
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513 | (12) |
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Chronic Granulomatous Disease |
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525 | (25) |
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Cell Adhesion and Leukocyte Adhesion Defects |
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550 | (15) |
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Cyclic and Congenital Neutropenia Due to Defects in Neutrophil Elastase |
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565 | (5) |
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570 | (8) |
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Inherited Hemophagocytic Syndromes |
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578 | (11) |
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Genevieve de Saint Basile |
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Genetically Determined Deficiencies of the Complement System |
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589 | (22) |
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Part III Assessment and Treatment |
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Assessment of the Immune System |
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611 | (22) |
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Genetic Aspects of Primary Immunodeficiencies |
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633 | (11) |
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Immunodeficiency Information Services |
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644 | (11) |
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Conventional Therapy of Primary Immunodeficiency Diseases |
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655 | (14) |
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Bone Marrow Transplantation for Primary Immunodeficiency Diseases |
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669 | (19) |
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688 | (19) |
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Index |
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707 | |