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Inborn Errors of Immunity and Phagocytosis

Monograph based upon Proceedings of the Fifteenth Symposium of The Society for the Study of Inborn Errors of Metabolism

  • Conference proceedings
  • © 1979

Overview

Editors:
  1. F. Güttler

    1. John F. Kennedy Institute, Glostrup, Denmark

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  2. J. W. T. Seakins

    1. Department of Chemical Pathology, Institute of Child Health, London

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  3. R. A. Harkness

    1. Division of Perinatal Medicine, MRC Clinical Research Centre, Harrow, Middlesex, London

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Table of contents (28 papers)

  1. Front Matter

    Pages i-xvi
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  2. Defects of Cell-mediated Immunity

    1. Front Matter

      Pages 1-1
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    2. Immunodeficiency diseases — a review

      • R. N. Pahwa, S. G. Pahwa, R. O’Reilly, Elizabeth M. Smithwick, R. A. Good
      Pages 3-68

    3. Genetics of the immune system

      • A. Svejgaard, E. Dickmeiss, G. S. Hansen, P. Platz, L. P. Ryder, M. Thomsen
      Pages 69-85

  3. Enzyme Defects and Immunodeficiency

    1. Front Matter

      Pages 87-87
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    2. Inborn errors of specific immunity: adenosine deaminase deficiency and purine nucleoside phosphorylase deficiency

      • H. J. Meuwissen, K. Parker, B. Cook
      Pages 89-104

    3. Adenosine deaminase deficiency: enzyme replacement therapy and investigations of the biochemical basis of immunodeficiency

      • S. H. Polmar
      Pages 105-120

    4. Prenatal diagnosis and heterozygote detection in adenosine deaminase deficiency

      • Rochelle Hirschhorn
      Pages 121-128

    5. Pathogenic mechanisms in deficiencies of adenosine deaminase and purine nucleoside phosphorylase

      • D. A. Carson, J. Kaye, J. E. Seegmiller
      Pages 129-135

    6. Purine nucleoside phosphorylase deficiency associated with cellular immunodeficiency: immunological studies during treatment

      • B. J. M. Zegers, J. W. Stoop, G. F. M. Hendrickx, S. K. Wadman, G. E. J. Staal
      Pages 137-145

    7. Purine nucleoside phosphorylase deficiency associated with cellular immunodeficiency: metabolic studies during treatment

      • S. K. Wadman, P. K. de Bree, G. F. M. Hendrickx, B. J. M. Zegers, J. W. Stoop
      Pages 147-154

    8. Severe combined immunodeficiency with B lymphocytes: a selective defect of precursor T cells

      • C. Griscelli, A. Durandy, F. Daguillard
      Pages 155-165

    9. Purine metabolism and the control of lymphocyte proliferation. Effects of exogenous adenosine on normal human lymphocytes

      • T. Hovi
      Pages 167-172

    10. Activity of adenosine deaminase and purine nucleoside phosphorylase in lymphocytes of man, horse and cattle

      • W. J. M. Tax, J. H. Veerkamp
      Pages 173-180

    11. Ageing and activities of purine metabolizing enzymes in leukocytes

      • J. Mejer, P. Nygaard
      Pages 181-187

    12. Immune function in Down’s syndrome

      • B. Björkstén, O. Bäck, B. Hägglöf, A. Tärnvik
      Pages 189-198
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Keywords

About this book

The rapid growth of immunology has greatly increased our understanding of disease; this growth has also generated a subject which at times appears separated from some of the basic medical sciences. Recent studies in the areas of purine metabolism and of polymorphonuclear neutrophil phago­ cyte function have, however, linked immunology and clinical medicine with biochemistry. The precise defects of the inborn errors of metabolism have now provided good evidence for the importance of purine metabolism specifically the enzymes adenosine deaminase and nucleoside phosphorylase in lymphocyte function. In view of this and the steady advance of clinical and biochemical investigation of the polymorphonuclear neutrophil phago­ cyte, it appeared timely to review the inborn errors of immunity and phagocytosis at the fifteenth annual symposium of the Society for the Study of Inborn Errors of Metabolism at Elsinore, Denmark on September II-14th, I~77. The papers presented at that meeting form the basis of this volume which brings together contributions from immunologists, biochemists and clinicians. This interdisciplinary communication should be helpful to those concerned with immune function in their patients or in the laboratory. The book is divided into four sections, One: defects of cell-mediated immunity, Two: enzyme defects and immunodeficiency, Three: disorders of non-specific immunity and Four: screening for immunodeficiency. Section One contains two reviews, one on immunodeficiency from Robert Good's group in New York and another on the genetics of the immune system from Arne Svejgaard of Copenhagen.

Editors and Affiliations

  • John F. Kennedy Institute, Glostrup, Denmark

    F. Güttler

  • Department of Chemical Pathology, Institute of Child Health, London

    J. W. T. Seakins

  • Division of Perinatal Medicine, MRC Clinical Research Centre, Harrow, Middlesex, London

    R. A. Harkness

Bibliographic Information

  • Book Title: Inborn Errors of Immunity and Phagocytosis

  • Book Subtitle: Monograph based upon Proceedings of the Fifteenth Symposium of The Society for the Study of Inborn Errors of Metabolism

  • Editors: F. Güttler, J. W. T. Seakins, R. A. Harkness

  • DOI: https://doi.org/10.1007/978-94-011-6197-8

  • Publisher: Springer Dordrecht

  • eBook Packages: Springer Book Archive

  • Copyright Information: The Society for the Study of Inborn Errors of Metabolism 1979

  • Softcover ISBN: 978-94-011-6199-2Published: 13 February 2012

  • eBook ISBN: 978-94-011-6197-8Published: 06 December 2012

  • Edition Number: 1

  • Number of Pages: 391

  • Topics: Pediatrics, Internal Medicine

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