| Description |
1 online resource (xvii, 726 pages, 32 unnumbered pages of plates) : illustrations (some color) |
| Contents |
Genetically determined immunodeficiency diseases / C.I. Edvard Smith, Hans D. Ochs, and Jennifer M. Puck -- Genetic principles and technologies in the study of immune disorders / Jennifer M. Puck and Robert L. Nussbaum -- Mammalian hematopoietic development and function / Gerald J. Sprangrude -- T cell development / Rae S.M. Yeung [and others] -- Molecular mechanisms guiding B cell development / Antonius Rolink [and others] -- Signal transduction by T and B lymphocyte antigen receptors / Neetu Gupta, Anthony L. DeFranco, and Arthur Weiss -- Lymphoid organ development, cell trafficking, and lymphocute responses / Sirpa Jalkanen and Marko Salmi -- Phagocytic system / Kuender D. Yang, Paul G. Quie, and Harry R. Hill -- X-linked severe combined immunodeficiency / Jennifer M. Puck -- Autosomal recessive severe combined immunodeficiency due to defects of cytokine signaling pathways / Fabio Candotti and Luigi Notarangelo -- V(D)J recombination defects / Jean-Pierre de Villartay, Klaus Schwarz, and Anna Villa -- Immunodeficiency due to defects of purine metabolism / Rochelle Hirschhorn, Fabio Candotti -- Severe combined immunodeficiency due to mutations in the CD45 gene / Talal Chatila and Markku Heikinheimo -- Severe combined immunodeficiency due to defects in T cell receptor-associated protein kinases / Melissa E. Elder -- Human interleukin-2 receptor [alpha] deficiency / Chaim M. Roifman -- CD3 and CD8 deficiencies / José R. Regueiro and Teresa Espanol -- Molecular basis of major histocompatibility complex class II deficiency / Walter Reith, Barbara Lisowska-Grospierre, and Alain Fischer -- Peptide transporter defects in human leukocyte antigen class I deficiency / Henri de la Salle, Lionel Donato, and Daniel Hanau -- CD40, CD40 ligand, and the hyper-IgM syndrome / Raif S. Geha, Alessandro Plebani, and Luigi Notarangelo -- Autosomal hyper-IgM syndromes caused by an intrinsic B cell defect / Anne Durandy, Patrick Revy, and Alain Fischer -- X-linked agammaglobulinemia / C.I. Edvard Smith, Anne B. Satterthwaite, and Owen N. Witte -- Autosomal recessive agammaglobulinemia / Mary Ellen Conley -- Genetic approach to common variable immunodeficiency and IgA deficiency / Lennart Hammarström and C.I. Edvard Smith -- Autoimmune lymphoproliferative syndrome / Jennifer M. Puck [and others] |
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Autoimmune polyendocrinopathy, candidiasis and ectodermal dystrophy / Leena Peltonen-Palotie, Maria Halonen, and Jaakko Perheentupa -- Immune dysregulation, polyendocrinopathy, enteropathy; X-linked inheritance / Troy R. Torgerson [and others] -- Periodic fever syndromes / Daniel Kastner, Susannah Brydges, and Keith M. Hull -- Inherited disorders of the interleukin-12/23-interferon gamma axis / Melanie Newport [and others] -- Ataxia-telangiectasia / Martin F. Lavin and Yosef Shiloh -- Chromosomal instability syndromes other than ataxia-telangiectasia / Rolf-Dieter Wegner [and others] -- Wiskott-Aldrich syndrome / Hans D. Ochs, Fred S. Rosen |
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X-linked lymphoproliferative disease due to defects of SH2D1A / Volker Schuster and Cox Terhorst -- DiGeorge syndrome / Deborah A. Driscoll and Kathleen E. Sullivan -- Hyper IgE recurrent infection syndromes / Bodo Grimbacher, Jennifer F. Puck, and Stevn M. Holland -- Immunodeficiency with centromere instability and facial anomalies / R. Scott Hansen, Corry Weemaes, and Cisca Wijmenga -- Immunodeficiencies with associated manifestations of skin, hair, teeth, and skeleton / Mario Abinun, Ilkka Kaitila and Jean-Laurent Casanova -- Chronic granulomatous disease / Dirk Roos, Taco W. Kuijpers, and John T. Curnutte -- Cell adhesion and leukocyte adhesion defects / Amos Etzioni and John M. Harlan |
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Cyclic and congenital neutropenia due to defects in neutrophil elastase / David Dale and Andrew Aprikyan -- Chediak-Higashi syndrome / Richard A. Spritz -- Inherited hemophagocytic syndromes / Geneviève de Saint Basile -- Genetically determined deficiencies of the complement system / Kathleen E. Sullivan and Jerry A. Winkelstein -- Assessment of the immune system / Helen M. Chapel, Siraj Misbah, and A. David Webster -- Genetic aspects of primary immunodeficiencies / Jennifer M. Puck -- Immunodeficiency information systems / Jouni Väliaho [and others] -- Conventional therapy of primary immunodeficiency diseases / E. Richard Stiehm and Helen M. Chapel -- Bone marrow transplantation for primary immunodeficiency diseases / Rebecca H. Buckley and Alain Fischer -- Gene therapy / Fabio Candotti and Alain Fischer |
| Summary |
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. The book should appeal to geneticists, pediatricians, immunologists, infectio |
| Bibliography |
Includes bibliographical references and index |
| Notes |
English |
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Print version record |
| Subject |
Immunological deficiency syndromes -- Genetic aspects
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Immunologic Deficiency Syndromes -- genetics
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MEDICAL -- Immunology.
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HEALTH & FITNESS -- Allergies.
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HEALTH & FITNESS -- Diseases -- Immune System.
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| Form |
Electronic book
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| Author |
Ochs, Hans D., 1936-
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Smith, C. I. Edvard, 1951-
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Puck, Jennifer, 1949-
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| LC no. |
2005027951 |
| ISBN |
9780199747962 |
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0199747962 |
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019514774X |
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9780195147742 |
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9786610564279 |
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6610564272 |
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1429420456 |
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9781429420457 |
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