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Pediatric Nephrology
Erschienen in: Pediatric Nephrology 5/2019

28.01.2019 | Editorial

Optimal duration of treatment with eculizumab in atypical hemolytic uremic syndrome (aHUS)—a question to be addressed in a scientific way

verfasst von: Gema Ariceta

Erschienen in: Pediatric Nephrology | Ausgabe 5/2019

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Excerpt

Atypical hemolytic uremic syndrome (aHUS) is an ultrarare disease caused, in most cases, by dysregulation of the alternative complement (C′) pathway at the surface of the vascular endothelium [1‐3]. Uncontrolled C′ activation due to genetic abnormalities of several regulatory proteins, such as complement factor H (FH) [4‐13] or auto-antibodies against FH synthesis [14, 15], lead to increased C3 convertase production and/or reduced degradation [16], which in turn result in excessive C5 activation, C5b-9 formation (also named terminal complement complex (TCC)), and subsequently endothelial cell lysis [17, 18]. Independent of the individual specific C′ anomaly, aHUS is a disease driven by a common pathogenic mechanism: C5 activation [19, 20]. …
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Metadaten
Titel
Optimal duration of treatment with eculizumab in atypical hemolytic uremic syndrome (aHUS)—a question to be addressed in a scientific way
verfasst von
Gema Ariceta
Publikationsdatum
28.01.2019
Verlag
Springer Berlin Heidelberg
Erschienen in
Pediatric Nephrology / Ausgabe 5/2019
Print ISSN: 0931-041X
Elektronische ISSN: 1432-198X
DOI
https://doi.org/10.1007/s00467-019-4192-7

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