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Immundefekte mit selektiver Vulnerabilität für einzelne Erreger

Immune defects with predisposition to selected pathogens

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Zusammenfassung

Klassische angeborene Immundefekte prädisponieren zu wiederholten, oft polytopen schweren Infektionen durch multiple opportunistische Erreger. In den letzten 10 Jahren sind auch genetisch bedingte Immundefekte mit selektiver Anfälligkeit für Infektionen durch einzelne Erregergruppen oder einzelne Erreger identifiziert worden. Hierzu gehören Immundefekte mit Prädisposition für Infektionen durch Mykobakterien und Salmonellen, für invasive bakterielle Infektionen, für Herpes-simplex-Enzephalitis, für durch Epstein-Barr-Virus induzierte lymphoproliferative Krankheitsbilder und für schwere Infektionen durch Papillomaviren. Die folgende Übersicht stellt Genetik, Pathophysiologie, klinisches Bild, Differenzialdiagnosen, diagnostisches Vorgehen, Prognose und Therapieoptionen dieser „neuen“ Immundefekte dar.

Abstract

Classical primary immunodeficiencies predispose to multiple and recurrent infections by multiple pathogens. During the last decade several“new” primary immunodeficiencies with selected predisposition to severe infections with mycobacteria and salmonellae, pyogenic bacteria, Epstein-Barr virus, papillomavirus and herpes simplex virus were identified. This review focuses on the pathophysiology, clinical presentation, a rational diagnostic approach and therapeutic options for these newly described primary immunodeficiencies.

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  1. Universitätsklinik und Poliklinik für Kinder und Jugendliche, Leipzig, Deutschland

    V. Schuster

  2. Klinik für Kinderheilkunde mit Schwerpunkt Pneumologie und Immunologie der Charité, Campus Rudolf Virchow, Augustenburger Platz 1, 13353, Berlin, Deutschland

    H. von Bernuth

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Schuster, V., von Bernuth, H. Immundefekte mit selektiver Vulnerabilität für einzelne Erreger. Monatsschr Kinderheilkd 157, 886–895 (2009). https://doi.org/10.1007/s00112-009-1989-7

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