Primary Immunodeficiency Diseases: A Molecular And Cellular Approach

Hardcover | August 23, 2006

byHans D. Ochs, C. I. Edward Smith, Jennifer M. Puck

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The second edition of Primary Immunodeficiency Diseases presents discussions of gene identification, mutation detection, and clinical and research applications for over 100 genetic immune disorders--disorders featuring an increased susceptibility to infections and, in certain conditions, anicreased rate of malignancies and autoimmune disorders. Since the publication of the first edition, a flurry of new disease entities has been defined and new treatment regimens have been introduced, the most spectacular being successful treatment by gene therapy for two genotypes of combinedimmunodeficiency. The first edition marked a historic turning point in the field of immunodeficiencies, demonstrating that many of the disorders of the immune systam could be understood at a molecular level. This new edition can proudly document the tremendous pace of progress in dissecting thecomplex immunologic networks responsible for protecting individuals from these disorders.

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The second edition of Primary Immunodeficiency Diseases presents discussions of gene identification, mutation detection, and clinical and research applications for over 100 genetic immune disorders--disorders featuring an increased susceptibility to infections and, in certain conditions, anicreased rate of malignancies and autoimmune d...

Hans D. Ochs is at University of Washington School of Medicine, Seattle. C. I. Edward Smith is at Novum, Karolinska Institute, Huddinge.

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Format:HardcoverDimensions:776 pages, 8.82 × 10.98 × 2.09 inPublished:August 23, 2006Publisher:Oxford University PressLanguage:English

The following ISBNs are associated with this title:

ISBN - 10:019514774X

ISBN - 13:9780195147742

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Table of Contents

1. C.I. Edward Smith, Hans D. Ochs, and Jennifer M. Puck: Genetically Determined Immunodeficiency Diseases: A Perspective2. Jennifer M. Puck and Robert L. Nussbaum: Genetic Principles and Technologies in the Study of Immune Disorders3. Gerald J. Sprangrude: Mammalian Hematopoietic Development and Function4. Rae S. M. Yeung, Josef M. Penninger, and Tak W. Mak: T-Cell Development5. Antonius Rolink, Jan Andersson, Ulf Grawunder, and Fritz Melchers: Molecular Mechanisms Guiding B-Cell Development6. Neetu Gupta, Anthony L. DeFranco, and Arthur Weiss: Signal Transduction by T and B Lymphocyte Antigen Receptors7. Sirpa Jalkanen and Marko Salmi: Regulation of Lymphocyte Responses, Cell Trafficking and Lymphoid Organ Development8. Kuender D. Yang, Anthony Segal, Harry R. Hill: The Phagocytic SystemPart II Syndromes. 9. Jennifer M. Puck: X-linked Severe Combined Immunodeficiency10. Fabio Candotti and Luigi Notarangelo: Autosomal Recessive SCID Due to Defects of Cytokine Signaling Pathways11. Anna Villa, Klaus Schwarz, and , J.P. Villartay: Recombination Defects12. Rochelle Hirschhorn, Fabio Candotti: Immunodeficiency Due to Defects of Purine Metabolism13. Markku Heikinheimo and Talal Chatila: CD45 Deficiency14. Melissa E. Elder: SCID Due to Defects in T-Cell-Receptor-Associated Protein Kinases15. Chaim M. Roifman: Human Interleukin-2 Receptor Alpha Deficiency16. Jose R. Regueiro Teresa Espanol et al.: CD3 and CD8 Deficiencies17. Walter Reith, Barbara Lisowska-Grospierre, and Alain Fischer: Molecular Basis of Major Histocompatibility Complex Class II Deficiency18. Henri de la Salle, Lionel Donato, Daniel Hanau, and Marie-Marthe Tongio: Peptide Transporter Defects in HLA Class I Deficiency19. Raif S. Geha, Alessandro, and Luigi Notarangelo: CD40, CD40 Ligand, and theHyper-IgM Syndrome20. Anne Durandy, Patrick Revy, and Alain Fischer: Autosomal Hyper-IgM Syndromes Caused by an Intrinsic B-cell Defect21. C.I. Edvard Smith and Owen N. Witte: X-linked Agammaglobulinemia: A Defect of Btk Tyrosine Kinase22. Mary Ellen Conley: Autosomal Recessive Agammaglobulinemia23. Lennart Hammarstrm and C.I. Edvard Smith: Genetic Approach to Common Variable Immunodeficiency and IgA Deficiency24. Jennifer M. Puck, Stephen E. Straus, Francoise Le Deist, Frederic Rieux-Laucat: Defects in Apoptosis with Autoimmune Manifestations25. Leena Peltonen, Maria Halonen, and Jaakko Perheentupa: Autoimmune Polyendocrinopathy, Candidiasis and Ectodermal Dystrophy (APECED)26. Hans D. Ochs, Mary E. Brunkow, and Steve Siegler: Immune Dysregulation, Polyendocrinopathy, Enteropathy; X-linked (IPEX)27. Daniel Kastner, Susannah Brydges, and Keith M. Hull: Periodic Fever Syndromes28. Melanie Newport, Steve Holland, Michael Levin, and Jean-Laurent Casanova: Inherited Disorders of the Interleukin-12/23-Interferon Gamma Axis29. Martin F. Lavin and Yosef Shiloh: Ataxia-Telangiectasia30. Karl Sperling, Markus Stumm, James German, Fanconi expert (e.g. Johnson Liu/French woman/Dutch): DNA Breakage and Repair Associated Syndromes Other Than Ataxia-Telangiectasia31. Hans D. Ochs, Fred S. Rosen: The Wiskott-Aldrich Syndrome32. Volker Schuster and Cox Terhorst: X-linked Lymphoproliferative Disease (XLP) due to Defects of SH2D1A33. Deborah A. Driscoll and Kathleen E. Sullivan: DiGeorge Syndrome: A Chromosome 22q11.2 Deletion Syndrome34. Bodo Grimbacher and Stevn M. Holland: Hyper IgE Recurrent Infection Syndromes35. Scott Hansen, Corry Weemaes, and Cisca Wijmenga: Immunodeficiency with Centromere Instability and Facial Anomalies36. Mario Abinun, Ilkka Kaitila and Jean-Laurent Casanova: Immunodeficiencies with Associated Manifestations of Skin, Hair, Teeth, and Skeleton37. Dirk Roos, Taco W. Kuijpers, and John T. Curnutte: Chronic Granulomatous Disease38. Amos Etzioni and John M. Harlan: Cell Adhesion and Leukocyte Adhesion Defects39. David Dale and Andrew Aprikyan: Cyclic and Congenital Neutropenia Due to Defects in Neutrophil Elastase40. Richard A. Spritz: Chediak-Higashi Syndrome41. Genevive de Saint Basile: Inherited Hemophagocytic Syndromes42. Kathleen E. Sullivan and Jerry A. Winkelstein: Genetically Determined Deficiencies of the Complement SystemPart III Assessment and Treatment. 43. Helen M. Chapel, Siraj Misbah, and David Webster: Assessment of the Immune System44. Jennifer M. Puck: Genetic Aspects of Primary Immunodeficiencies45. Jouni Valiaho, Crina Samarghitean, Hilkka Piirila, Marianne Pusa, and Mauno Vihinen: Immunodeficiency Information Systems46. E. Richard Stiehm and Helen M. Chapel: Conventional Therapy of Primary Immunodeficiency Diseases47. Rebecca H. Buckley and Alain Fischer: Bone Marrow Transplantation for Primary Immunodeficiency Diseases48. Fabio Candotti and Alain Fischer: Gene Therapy

Editorial Reviews

"This latest edition of Primary Immunodeficiency Diseases emphasized the molecular bases of these disorders, but there are also thorough discussions of their clinical aspects, and these will make the book an important addition to the library of any physician who treats patietns with primaryimmunodeficiencies."--New England Journal of Medicine