Nierenbeteiligung bei thrombotischer Mikroangiopathie

ZUSAMMENFASSUNG

Der Begriff thrombotische Mikroangiopathie (TMA) fasst eine Gruppe von Krankheiten zusammen, bei denen sich durch unterschiedliche Mechanismen Mikrothromben im Kapillarbett und in den kleinen Gefäßen bilden. Diese lösen eine mechanische Hämolyse mit Anämie und (Verbrauchs-)Thrombopenie aus. Die Mikrozirkulation wird beeinträchtigt und es kommt zu einer Ischämie mit Funktionsstörung der betroffenen Organe, inklusive der Niere. Das zentrale Nervensystem, Herz, Lunge und Gastrointestinaltrakt können ebenso beteiligt sein. Die häufigste TMA im Kindesalter ist das Shiga-Toxin assoziierte hämolytisch-urämische Syndrom (STEC-HUS). Das atypische hämolytisch-urämische Syndrom (aHUS) und die thrombotisch-thrombozytopenische Purpura (TTP) sind in dieser Altersgruppe wesentlich seltener. Bei Erwachsenen treten mit zunehmendem Alter überwiegend sekundäre TMA-Formen auf.

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