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Inborn errors of specific immunity: adenosine deaminase deficiency and purine nucleoside phosphorylase deficiency

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Inborn Errors of Immunity and Phagocytosis

Abstract

Many inborn errors of metabolism are known to occur in immune defence systems. Most of these involve non-specific immunity, particularly the complement and neutrophil granulocyte systems. Inborn errors of specific immunity were not described until recently. In this discussion, we will review the two most important diseases in this latter group, deficiency of adenosine deaminase (ADA) and purine nucleoside phosphorylase (PNP). Lack of transcobalamine II, leading to hypogammaglobulinaemia, may represent a third disorder in this group1 but we will not consider this entity here.

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Authors
  1. H. J. Meuwissen
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  2. K. Parker
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  3. B. Cook
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Editor information

Editors and Affiliations

  1. John F. Kennedy Institute, DK-2600, Glostrup, Denmark

    F. Güttler

  2. Department of Chemical Pathology, Institute of Child Health, WC1N 1EH, London

    J. W. T. Seakins

  3. Division of Perinatal Medicine, MRC Clinical Research Centre, Harrow, HA1 3UJ, Middlesex, London

    R. A. Harkness

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© 1979 The Society for the Study of Inborn Errors of Metabolism

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Meuwissen, H.J., Parker, K., Cook, B. (1979). Inborn errors of specific immunity: adenosine deaminase deficiency and purine nucleoside phosphorylase deficiency. In: Güttler, F., Seakins, J.W.T., Harkness, R.A. (eds) Inborn Errors of Immunity and Phagocytosis. Springer, Dordrecht. https://doi.org/10.1007/978-94-011-6197-8_3

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  • DOI: https://doi.org/10.1007/978-94-011-6197-8_3

  • Publisher Name: Springer, Dordrecht

  • Print ISBN: 978-94-011-6199-2

  • Online ISBN: 978-94-011-6197-8

  • eBook Packages: Springer Book Archive

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