Hämolytisch-urämisches Syndrom im Kindes- und Jugendalter View Full Text


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Article Info

DATE

2017-11

AUTHORS

J. Holle, B. Lange-Sperandio, C. Mache, J. Oh, L. Pape, F. Schaefer, U. Vester, L. T. Weber, D. Müller

ABSTRACT

Das hämolytisch-urämische Syndrom (HUS) ist definiert durch die Trias mikroangiopathische hämolytische Anämie, Thrombozytopenie und akute Nierenfunktionseinschränkung. Die häufigste Form im Kindesalter ist das STEC-HUS, bei dem eine Infektion mit shigatoxinproduzierenden Erregern zur hämorrhagischen Enterokolitis führt. In etwa 10 % der Fälle entsteht daraus ein HUS. Extrarenale Symptome können vorkommen. Die Diagnosesicherung erfolgt durch entsprechende Stuhl- bzw. serologische Diagnostik. Eine spezifische Therapie ist nicht möglich. Beim komplementvermittelten HUS kommt es durch Mutationen, Antikörperbildung und Trigger-Ereignisse zur Dysregulation im alternativen Weg der Komplementaktivierung. Aufgrund der spezifischen Therapiemöglichkeit mit dem C5-Inhibitor Eculizumab hat sich die Prognose deutlich gebessert. In seltenen Fällen lösen Infektionen (z. B. Pneumokokken) oder Grunderkrankungen (z. B. Cobalamin-C-Mangel) ein HUS aus. Aufgrund der Komplexität von Diagnostik und Therapie wird die Behandlung in einem kindernephrologischen Zentrum empfohlen. More... »

PAGES

1005-1018

Identifiers

URI

http://scigraph.springernature.com/pub.10.1007/s00112-017-0331-z

DOI

http://dx.doi.org/10.1007/s00112-017-0331-z

DIMENSIONS

https://app.dimensions.ai/details/publication/pub.1090591494


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34 schema:description Das hämolytisch-urämische Syndrom (HUS) ist definiert durch die Trias mikroangiopathische hämolytische Anämie, Thrombozytopenie und akute Nierenfunktionseinschränkung. Die häufigste Form im Kindesalter ist das STEC-HUS, bei dem eine Infektion mit shigatoxinproduzierenden Erregern zur hämorrhagischen Enterokolitis führt. In etwa 10 % der Fälle entsteht daraus ein HUS. Extrarenale Symptome können vorkommen. Die Diagnosesicherung erfolgt durch entsprechende Stuhl- bzw. serologische Diagnostik. Eine spezifische Therapie ist nicht möglich. Beim komplementvermittelten HUS kommt es durch Mutationen, Antikörperbildung und Trigger-Ereignisse zur Dysregulation im alternativen Weg der Komplementaktivierung. Aufgrund der spezifischen Therapiemöglichkeit mit dem C5-Inhibitor Eculizumab hat sich die Prognose deutlich gebessert. In seltenen Fällen lösen Infektionen (z. B. Pneumokokken) oder Grunderkrankungen (z. B. Cobalamin-C-Mangel) ein HUS aus. Aufgrund der Komplexität von Diagnostik und Therapie wird die Behandlung in einem kindernephrologischen Zentrum empfohlen.
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