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Das hämolytisch-urämische Syndrom im Kindesalter

Hemolytic-uremic syndrome


Das hämolytisch-urämische Syndrom (HUS) ist mit die häufigste Ursache des akuten Nierenversagens im Kindesalter. Es besteht aus der Trias Coombs-negative akute Hämolyse, Thrombozytopenie und akutem Nierenversagen. Das HUS stellt eine heterogene Gruppe mit unterschiedlicher Pathogenese und Verlaufsform dar. Histologisch ist allen Formen die thrombotische Mikroangiopathie (TMA) gemeinsam. Aus therapeutischer und prognostischer Sicht hat es sich in der Pädiatrie bewährt, das HUS in das klassische, diarrhöassoziierte HUS (D+ HUS) und das atypische, nicht diarrhöassoziierte HUS (D− HUS) einzuteilen. Dabei liegt im Kindesalter in über 90% aller Fälle ein D+ HUS vor. Obwohl in den letzten Jahren zahlreiche Ursachen des D− HUS identifiziert werden konnten, bleibt ein großer Teil der Fälle pathogenetisch immer noch unklar. Während das D+ HUS eine insgesamt gute Prognose hat, ist die des D− HUS eher ungünstig. Im vorliegenden Beitrag werden die verschiedenen Formen des HUS im Hinblick auf Ätiologie, therapeutische Optionen und Prognose erläutert.


Hemolytic-uremic syndrome (HUS) is among the most common causes of acute renal failure in childhood. A heterogenous group of diseases with varying etiology and clinical course, HUS is characterized by Coombs-negative acute hemolysis, thrombocytopenia, and acute renal failure. Histologically, all forms have thrombotic microangiopathy in common. In terms of therapeutic options and prognostic factors, it is reasonable to differentiate between classic diarrhea-associated (D+) HUS and atypical HUS not associated with diarrhea (D− HUS). In childhood 90% of all cases are D+ HUS, with a more favorable prognosis, whereas the prognosis in D− HUS is rather poor. Although many underlying causes for different D− HUS forms have been identified in the last few years, for many cases the pathogenesis remains unclear. In the following we discuss etiology, prognosis, and therapeutic options for the different forms of HUS.

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Thumfart, J., Müller, D. Das hämolytisch-urämische Syndrom im Kindesalter. Nephrologe 3, 297–307 (2008).

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