Genetisch bedingte Defekte der angeborenen Immunität

Janine Reichenbach; Horst von Bernuth

doi:10.1055/s-0038-1630894

Kinder- und Jugendmedizin, Table of Contents

Kinder- und Jugendmedizin 2008; 08(03): 171-178
DOI: 10.1055/s-0038-1630894

Immundefekte

Schattauer GmbH

Genetisch bedingte Defekte der angeborenen Immunität

Erhöhte Anfälligkeit für mykobakterielle Infektionen, für invasive bakterielle Infektionen und für Herpes-simplex-EnzephalitisGenetic defects of the innate immunity that cause an increased susceptibility for mycobacterial infections, invasive pyogenic infections and herpes simplex encephalitis

Janine Reichenbach

¹Universitäts-Kinderspital Zürich, Abteilung Immunologie/Hämatologie/KMT (Leiter: Prof. Dr. med. R. A. Seger)

,

Horst von Bernuth

²Charité Universitätsmedizin Berlin, Campus Virchow-Klinikum, Klinik für Pädiatrie m. S. Pneumologie und Immunologie (Leiter: Prof. Dr. med. U. Wahn)

› Author Affiliations

Abstract

Zusammenfassung

Bei Patienten mit wenigen Infektionen, mit Infektionen durch nicht opportunistische Erreger und mit Infektionen durch nur einen bestimmten Erreger muss auch an einen Immundefekt gedacht werden. In diesem Beitrag werden Immundefekte vorgestellt, die durch Störungen in Signalwegen der angeborenen Immunität zu einer spezifischen Anfälligkeit gegen eine bestimmte Erregergruppe oder nur einen Erreger führen. Eine besondere Anfälligkeit für Mykobakterien kann ein Hinweis für Defekte in den Signalwegen von Interferon-γ und Interleukin-12 sein. Defekte der MyD88- und IRAK-4-abhängigen Signalwege, der Tollähnlichen und Interleukin-1-ähnlichen Rezeptoren prädisponieren zu invasiven bakteriellen Infektionen durch insbesondere Staphylococcus aureus und Streptococcus pneumoniae. Patienten mit sporadischer Enzephalitis durch Herpes-simplex-Virus-1 sollten auf Defekte der TLR3/UNC93B abhängigen Signalwege untersucht werden.

Summary

In patients with few infections, infections caused by nonopportunistic pathogens and infections caused by only a single pathogen an immunodeficiency must also be considered. We here present immunodeficiencies that are caused by impaired signalling in pathways of the innate immune system. These defects predispose to a specific susceptibility to one group of pathogens or only one pathogen. An increased susceptibility to mycobacterial infections can indicate a defect in the Interferon-γ and Interleukin-12 dependent pathways. Defects in MyD88 and IRAK-4-dependent pathways of Toll-like and Interleukin-1 receptors predispose to invasive pyogenic infections, in particular by Staphylococcus aureus and Streptococcus pneumoniae. In patients with herpes simplex encephalitis defects of TLR3/UNC93B-dependent signalling should be suspected.

Schlüsselwörter

Mykobakterielle Infektionen - invasive bakterielle Infektionen - Herpes-simplex-Enzephalitis - IFN-γ-/Interleukin-12-System - Toll-ähnliche/Interleukin-1-Rezeptoren

Keywords

Mycobacterial disease - invasive pyogenic bacteria - herpes simplex encephalitis - IFN-γ/Interleukin-12 system - Tolllike/Interleukin-1 receptors

Full Text

References

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