학술논문
An Ex Vivo Test of Complement Activation on Endothelium for Individualized Eculizumab Therapy in Hemolytic Uremic Syndrome
Document Type
Article
Author
Galbusera, Miriam; Noris, Marina; Gastoldi, Sara; Bresin, Elena; Mele, Caterina; Breno, Matteo; Cuccarolo, Paola; Alberti, Marta; Valoti, Elisabetta; Piras, Rossella; Donadelli, Roberta; Vivarelli, Marina; Murer, Luisa; Pecoraro, Carmine; Ferrari, Elisa; Perna, Annalisa; Benigni, Ariela; Portalupi, Valentina; Remuzzi, Giuseppe
Source
American Journal of Kidney Diseases; July 2019, Vol. 74 Issue: 1 p56-72, 17p
Subject
Language
ISSN
02726386; 15236838
Abstract
Although primary atypical hemolytic uremic syndrome (aHUS) is associated with abnormalities in complement genes and antibodies to complement factor H, the role of complement in secondary aHUS remains debatable. We evaluated the usefulness of an ex vivo test to: (1) detect complement activation within the endothelium in primary and secondary aHUS, (2) differentiate active disease from remission, (3) monitor the effectiveness of eculizumab therapy, and (4) identify relapses during eculizumab dosage tapering and after discontinuation of treatment.