Pathogenese und Diagnostik von AA-Amyloidosen
Tóm tắt
AA-Amyloidosen sind mit einer hohen Morbidität und Mortalität verbunden. Der Krankheit liegen chronisch entzündliche Prozesse zugrunde, wobei rheumatische Erkrankungen in Europa führend sind. Die Bildung von AA-Amyloid setzt eine anhaltende Produktion des Akutphase-Proteins Serum-Amyloid A (SAA) voraus. Dieses physiologische Protein unterliegt Störungen der Faltung, wodurch es zur Aggregation und zur Verbindung mit der Serum-Amyloid-P-Komponente kommt. SAA und SAP bilden gemeinsam mit Proteoglykanen schwer lösliche Amyloidfibrillen, die zu strukturellen Gewebestörungen, aber auch zu direkten zellulären Effekten führen. Die renale Krankheitsmanifestation der Amyloidosen ist durch eine Proteinurie gekennzeichnet, die sich häufig als nephrotisches Syndrom darstellt. Bei anamnestischem und klinischem Anhalt für eine AA-Amyloidose erfolgt die Sicherung aus dem betroffenen Gewebe mittels Kongorotfärbung und Mikroskopie unter polarisiertem Licht, sowie dem immunhistochemischen Nachweis von SAA.
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