Article
Mutation of complement factor B causing massive fluid-phase dysregulation of the alternative complement pathway can result in atypical hemolytic uremic syndrome.
Kidney international - 1 Nov 2020
Zhang Yuzhou, Kremsdorf Robin A, Sperati C John, Henriksen Kammi J, Mori Mari, Goodfellow Renee X, Pitcher Gabriella R, Benson Cindy L, Borsa Nicolo Ghiringhelli, Taylor Ronald P, Nester Carla M, Smith Richard J H
Abstract excerpt
Atypical hemolytic uremic syndrome is an ultra-rare disease characterized by microangiopathic hemolytic anemia, thrombocytopenia and acute kidney injury. Its pathogenesis is driven most frequently by dysregulated cell-surface control of the alternative pathway of complement secondary to inherited and/or acquired factors. Here we evaluated two unrelated patients with atypical hemolytic uremic syndrome. The first,...
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