图书简介
This book will present the principles of immunology, genetics, and molecular biology as they pertain to the primary immunodeficiency diseases; the hallmark of these diseases is an increased susceptbility to infections. The major authorities in the field address both the scientific aspects and the practical, clinical aspects of these disorders, including therapeutic strategies. The second edition features 11 new chapters as well as a significant updating of all the first edition material in this fast-developing field.
Part I Overview; 1: C.I. Edvard Smith, Hans D. Ochs, and Jennifer M. Puck: Genetically Determined Immunodeficiency Diseases: A Perspective; 2: Jennifer M. Puck and Robert L. Nussbaum: Genetic Principles and Technologies in the Study of Immune Disorders; 3: Gerald J. Sprangrude: Mammalian Hematopoietic Development and Function; 4: Rae S. M. Yeung, Pamela S. Ohashi, Mary E. Saunders, and Tak W. Mak: T Cell Development; 5: Antonius Rolink, Jan Andersson, Ulf Grawunder, and Fritz Melchers: Molecular Mechanisms Guiding B Cell Development; 6: Neetu Gupta, Anthony L. DeFranco, and Arthur Weiss: Signal Transduction by T and B Lymphocyte Antigen Receptors; 7: Sirpa Jalkanen and Marko Salmi: Lymphoid Organ Development, Cell Trafficking, and Lymphocyte Responses; 8: Kuender D. Yang, Paul G. Quie, and Harry R. Hill: Phagocytic System; Part II Syndromes; 9: Jennifer M. Puck: X-linked Severe Combined Immunodeficiency; 10: Fabio Candotti and Luigi Notarangelo: Autosomal Recessive Severe Combined Immunodeficiency Due to Defects in Cytokine Signaling Pathways; 11: Jean-Pierre de Villartay, Klaus Schwarz, and Anna Villa: V(D)J Recombination Defects; 12: Rochelle Hirschhorn, and Fabio Candotti: Immunodeficiency Due to Defects of Purine Metabolism; 13: Tala A. Chatila and Markku Heikinheimo: Severe Combined Immunodeficiency Due to Mutations in the CD45 Gene; 14: Melissa E. Elder: Severe Combined Immunodeficiency Due to Defects in T Cell Receptor-Associated Protein Kinases; 15: Chaim M. Roifman: Human Interleukin-2 Receptor Alpha Deficiency; 16: Jose R. Regueiro and Teresa Espanol: CD3 and CD8 Deficiencies; 17: Walter Reith, Barbara Lisowska-Grospierre, and Alain Fischer: Molecular Basis of Major Histocompatibility Complex Class II Deficiency; 18: Henri de la Salle, Lionel Donato, and Daniel Hanau: Peptide Transporter Defects in Human Leukocyte Antigen Class I Deficiency; 19: Raif S. Geha, Alessandro Plebani, and Luigi D. Notarangelo: CD40, CD40 Ligand, and the Hyper-IgM Syndrome; 20: Anne Durandy, Patrick Revy, and Alain Fischer: Autosomal Hyper-IgM Syndromes Caused by an Intrinsic B Cell Defect; 21: C.I. Edvard Smith, Anne B. Satterthwaite, and Owen N. Witte: X-linked Agammaglobulinemia: A Disease of Btk Tyrosine Kinase; 22: Mary Ellen Conley: Autosomal Recessive Agammaglobulinemia; 23: Lennart Hammarstr and C.I. Edvard Smith: Genetic Approach to Common Variable Immunodeficiency and IgA Deficiency; 24: Jennifer M. Puck, Frederic Rieux-Laucat, Franise Le Deist, and Stephen E. Straus: Autoimmune Lymphoproliferative Syndrome; 25: Leena Peltonen-Palotie, Maria Halonen, and Jaakko Perheentupa: Autoimmune Polyendocrinopathy, Candidiasis, Ectodermal Dystrophy; 26: Troy R. Torgerson, Eleonora Gambineri, Steven F. Ziegler, and Hans D. Ochs : Immune Dysregulation, Polyendocrinopathy, Enteropathy, and X-Linked Inheritance; 27: Daniel L. Kastner, Susannah Brydges, and Keith M. Hull: Periodic Fever Syndromes; 28: Melanie J. Newport, Steven M. Holland, Michael Levin, and Jean-Laurent Casanova: Inherited Disorders of the Interleukin-12/23-Interferon Gamma Axis; 29: Martin F. Lavin and Yosef Shiloh: Ataxia-Telangiectasia; 30: Rolf-Dieter Wegner, James J. German, Krystyna H. Chrzanowska, Martin Digweed, and Markus Stumm: Chromosomal Instability Syndromes Other Than Ataxia-Telangiectasia; 31: Hans D. Ochs and Fred S. Rosen: Wiskott-Aldrich Syndrome; 32: Volker Schuster and Cox Terhorst: X-linked Lymphoproliferative Disease due to Defects of SH2D1A; 33: Deborah A. Driscoll and Kathleen E. Sullivan: DiGeorge Syndrome: A Chromosome 22q11.2 Deletion Syndrome; 34: Bodo Grimbacher, Jennifer M. Puck, and Steven M. Holland: Hyper-IgE Recurrent Infection Syndromes; 35: R. Scott Hansen, Corry Weemaes, and Cisca Wijmenga: Immunodeficiency with Centromere Instability and Facial Anomalies; 36: Mario Abinun, Ilkka Kaitila and Jean-Laurent Casanova: Immunodeficiencies with Associated Manifestations of Skin, Hair, Teeth, and Skeleton; 37: Dirk Roos, Taco W. Kuijpers, and John T. Curnutte: Chronic Granulomatous Disease; 38: Amos Etzioni and John M. Harlan: Cell Adhesion and Leukocyte Adhesion Defects; 39: David C. Dale and Andrew G. Aprikyan: Cyclic and Congenital Neutropenia Due to Defects in Neutrophil Elastase; 40: Richard A. Spritz: Chediak-Higashi Syndrome; 41: Genevie de Saint Basile: Inherited Hemophagocytic Syndromes; 42: Kathleen E. Sullivan and Jerry A. Winkelstein: Genetically Determined Deficiencies of the Complement System; Part III Assessment and Treatment; 43: Helen M. Chapel, Siraj Misbah, and David B. Webster: Assessment of the Immune System; 44: Jennifer M. Puck: Genetic Aspects of Primary Immunodeficiencies; 45: Jouni Viaho, Crina Samarghitean, Hilkka Piiril? Marianne Pusa, and Mauno Vihinen: Immunodeficiency Information Systems; 46: E. Richard Stiehm and Helen M. Chapel: Conventional Therapy of Primary Immunodeficiency Diseases; 47: Rebecca H. Buckley and Alain Fischer: Bone Marrow Transplantation for Primary Immunodeficiency Diseases; 48: Fabio Candotti and Alain Fischer: Gene Therapy
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