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]. …