3074 CHANGES IN THE CLINICAL CHARACTERISTICS AND MANAGEMENT OF PATIENTS WITH AHUS OVER 10 YEARS: TRENDS FROM THE GLOBAL AHUS REGISTRY

Abstract Background and Aims Atypical haemolytic uraemic syndrome (aHUS) is a rare disease predominantly caused by alternative complement pathway dysregulation. Prior to 2011, before the targeted complement inhibitor eculizumab became available, aHUS frequently led to end-stage kidney disease (ESKD)...

Ausführliche Beschreibung

Gespeichert in:
Bibliographische Detailangaben
Veröffentlicht in:Nephrology, dialysis, transplantation dialysis, transplantation, 2023-06, Vol.38 (Supplement_1)
Hauptverfasser: Siedlecki, Andrew, Al-Dakkak, Imad, Anokhina, Katerina, Ariceta, Gema, Ardissino, Gianluigi, Greenbaum, Laurence A, Licht, Christoph, Van de Walle, Johan
Format: Artikel
Sprache:eng
Online-Zugang:Volltext
Tags: Tag hinzufügen
Keine Tags, Fügen Sie den ersten Tag hinzu!
Beschreibung
Zusammenfassung:Abstract Background and Aims Atypical haemolytic uraemic syndrome (aHUS) is a rare disease predominantly caused by alternative complement pathway dysregulation. Prior to 2011, before the targeted complement inhibitor eculizumab became available, aHUS frequently led to end-stage kidney disease (ESKD) and early death. Treatment with eculizumab led to notable improvements in outcomes; whether patient characteristics and management has continued to change over time is unknown. Using data from the Global aHUS Registry, we assessed clinical characteristics and management of patients with aHUS over 10 years to identify any potential trends. Method All patients enrolled in the Global aHUS Registry from 2012–2022 were included. Patients were categorised according to age at aHUS onset (adult [≥18 years] vs paediatric [
ISSN:0931-0509
1460-2385
DOI:10.1093/ndt/gfad063d_3074