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Chronic Granulomatous Disease: An Update and a Paradigm for the Use of Interferon-γ as Adjunct Immunotheraphy in Infectious Diseases

  • Chapter
Macrophage Biology and Activation

Part of the book series: Current Topics in Microbiology and Immunology ((CT MICROBIOLOGY,volume 181))

Abstract

Chronic granulomatous disease (CGD) is a rare inherited disorder in which the phagocyte NADPH oxidase is disabled. The failure of the enzyme to generate superoxide and related oxygen intermediates renders the patients with this disease susceptible to recurrent bacterial and fungal infections. The clinical syndrome (reviewed Forrest et al. 1988) usually presents within the first years of life with a history of recurrent infections, mostly pneumonias, abscesses of the liver or lungs, or subcutaneous bacterial infections. Despite the use of high dose antibiotics, the phagocytes’ failure to kill ingested organisms serves as a stimulus for the chronic inflammatory state and granuloma formation. The granulomas are attempts to wall off infectious foci and if they occur in vital organs, like the gastrointestinal tract, kidney, liver, and brain, they contribute to the mortality and morbidity of this disorder.

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Authors and Affiliations

  1. Division of Hematology/Oncology, The Children’s Hospital and Dana-Farber Cancer Institute, Department of Pediatrics, Harvard Medical School, Boston, MA, 02115, USA

    R. A. B. Ezekowitz

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  1. R. A. B. Ezekowitz
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Editors and Affiliations

  1. University of Kansas Cancer Ctr., Kansas City, KA, 66160-7312, USA

    Stephen W. Russell (Wilkinson Distinguished Professor and Director) (Wilkinson Distinguished Professor and Director)

  2. Sir William Dunn School of Pathology, University of Oxford, South Parks Road, Oxford, OX1 3RE, UK

    Siamon Gordon

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© 1992 Springer-Verlag Berlin Heidelberg

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Ezekowitz, R.A.B. (1992). Chronic Granulomatous Disease: An Update and a Paradigm for the Use of Interferon-γ as Adjunct Immunotheraphy in Infectious Diseases. In: Russell, S.W., Gordon, S. (eds) Macrophage Biology and Activation. Current Topics in Microbiology and Immunology, vol 181. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-642-77377-8_11

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  • DOI: https://doi.org/10.1007/978-3-642-77377-8_11

  • Publisher Name: Springer, Berlin, Heidelberg

  • Print ISBN: 978-3-642-77379-2

  • Online ISBN: 978-3-642-77377-8

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