AUTHOR=Kollbrunner Lara , Hirt-Minkowski Patricia , Sanz Javier , Bresin Elena , Neuhaus Thomas J. , Hopfer Helmut , Jehle Andreas W. 

TITLE=Case Report: Lipoprotein Glomerulopathy Complicated by Atypical Hemolytic Uremic Syndrome

JOURNAL=Frontiers in Medicine

VOLUME=8

YEAR=2021

URL=https://www.frontiersin.org/journals/medicine/articles/10.3389/fmed.2021.679048

DOI=10.3389/fmed.2021.679048

ISSN=2296-858X

ABSTRACT=<p>Lipoprotein glomerulopathy (LPG) is a rare inherited disease caused by mutations in the APOE gene, encoding apolipoprotein E (apoE). Atypical hemolytic uremic syndrome (aHUS) is a thrombotic microangiopathy (TMA) characterized by overactivation of the alternative complement pathway. Here we report the case of a 21-year-old man with LPG who developed aHUS. A functional complement assay demonstrated an overactivation of the complement system. Complementary genetic analysis revealed a homozygous aHUS risk allele for complement factor-H related 1 (CFHR1), CFHR1<sup>*</sup>B. To the best of our knowledge, this is the first report of an aHUS in a patient with LPG.</p>