Hämolytisch-urämisches Syndrom

Zusammenfassung

Das typische, diarrhöassoziierte hämolytisch-urämische Syndrom (D-HUS) beruht auf einer Infektion mit Shigatoxin-bildenden enterohämorrhagischen Escherichia coli (EHEC). Shigatoxin provoziert durch Endothelschädigung und Schaffung eines inflammatorischen, prothrombotischen Milieus eine thrombotische Mikroangiopathie (TMA), die mit akutem Nierenversagen, neurologischen Funktionsstörungen und anderen Organmanifestationen einhergehen kann. Das D-HUS ist vom atypischen HUS (A-HUS) zu unterscheiden, dessen Ursache meist ein Defekt löslicher oder membranständiger Komplementregulatoren ist. Auch das A-HUS resultiert in einer akuten oder chronischen TMA mit ähnlichen Organmanifestationen. Eine 3. Erkrankung, die mit TMA einhergeht, ist die thrombotisch-thrombozytopenische Purpura (TTP), beruhend auf einem Defekt der VWF-spaltenden (VWF: von-Willebrand-Faktor) Protease ADAMTS13. Weitere Erkrankungen können eine TMA simulieren oder mit dieser einhergehen. Im Jahr 2006 wurde eine neue, stärker ätiologisch und pathophysiologisch orientierte Klassifikation von Erkrankungen mit TMA vorgeschlagen. Die in der Differenzialdiagnostik erforderlichen Untersuchungen sind aufwendig und zum Teil noch nicht flächendeckend verfügbar. Die Diagnostik von D-HUS, A-HUS und TTP ist deshalb in der Praxis schwierig, gewinnt wegen zunehmend differenzierter Therapieoptionen jedoch an Bedeutung. Dies gilt insbesondere für den Einsatz der Plasmapherese und des kürzlich zur Therapie des A-HUS zugelassenen Komplementinhibitors Eculizumab.

Key words

D-HUS - Shigatoxin - thrombotische Mikroangiopathie - A-HUS

• Literatur

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