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Komplementsystem und Komplementdefekte

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Pädiatrie

Part of the book series: Springer Reference Medizin ((SRM))

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Zusammenfassung

Das Komplementsystem ist ein wesentlicher Bestandteil im Netzwerk der körpereigenen Immunabwehr. Es besteht aus einer komplexen Kaskade von mehr als 30 verschiedenen Komponenten und Regulatoren. Zu seinen Hauptaufgaben zählt die direkte Zerstörung von Zellen und Erregern, die Opsonisierung von Fremdpartikeln und Immunkomplexen als Vorbedingung zu deren Phagozytose und die Aktivierung von Abwehrzellen des Immunsystems. Seine Leistungen sind permanent und sofort verfügbar. Dem System kommen somit besonders in der Frühphase einer Infektion, aber auch als Mediatorsystem im Bereich der akuten und chronischen Entzündungsreaktion, wichtige Aufgaben zu. Die pathophysiologische Bedeutung von Komplementstörungen liegt einerseits in den relativ selten vorkommenden Komplementdefizienzen, jedoch mehr noch in den Konsequenzen eines überaktivierten und/oder dysregulierten Systems als Basis für eine Vielzahl zum Teil schwerwiegender Entzündungserkrankungen, von Glomerulonephritiden bis hin zur Sepsis und Multi-Organversagen.

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Correspondence to Michael Kirschfink .

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Kirschfink, M. (2020). Komplementsystem und Komplementdefekte. In: Hoffmann, G.F., Lentze, M.J., Spranger, J., Zepp, F., Berner, R. (eds) Pädiatrie. Springer Reference Medizin. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-662-60300-0_101

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  • DOI: https://doi.org/10.1007/978-3-662-60300-0_101

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