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18-04-2024 | Hemolytic Uremic Syndrome | Editorial Commentary

Managing anti-factor H antibody-associated hemolytic uremic syndrome: time for consensus

Authors: Priyanka Khandelwal, Arvind Bagga

Published in: Pediatric Nephrology

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Excerpt

Atypical hemolytic uremic syndrome (aHUS) is an ultrarare disease affecting approximately 2 cases per 100,000 per year, of which children comprise 0.26–0.75 cases per 100,000 [1]. It is a rapidly progressive illness, frequently causing severe acute kidney injury requiring dialytic support and multisystem complications [2]. Dysregulation of the alternative complement pathway is key to its pathogenesis, either due to inherited genetic defects in ~ 20–40% of patients or acquired autoantibodies to factor H (anti-FH), the chief regulatory protein of the alternative pathway [3]. …
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Metadata
Title
Managing anti-factor H antibody-associated hemolytic uremic syndrome: time for consensus
Authors
Priyanka Khandelwal
Arvind Bagga
Publication date
18-04-2024
Publisher
Springer Berlin Heidelberg
Published in
Pediatric Nephrology
Print ISSN: 0931-041X
Electronic ISSN: 1432-198X
DOI
https://doi.org/10.1007/s00467-024-06374-w