Objective To describe the clinicopathological features, complement abnormalities, triggers,
treatment, and outcomes of C3 glomerulopathy. Patients and Methods A total of 114 patients …

Background Complement is a key component of the innate immune system, and variation in
genes that regulate its activation is associated with renal and other disease. We aimed to …

Factor I (FI) is the major complement inhibitor that degrades C3b and C4b in the presence of
cofactors such as factor H (FH) and membrane cofactor protein (MCP). Recently, mutations …

Fifty children with idiopathic membranoproliferative glomerulonephritis (MPGN), aged 2–14
years at apparent onset, were monitored for the presence of C3 nephritic factor (C3 NeF) …

Many of the complement regulatory genes within the RCA cluster (1q32) have arisen
through genomic duplication and the resulting high degree of sequence identity is likely to …

Methods All patients and controls were Caucasian. Forty-eight pa-tients with IgA
nephropathy and 19 patients with HSP were evaluated in Lexington, Kentucky. All patients …

Atypical hemolytic uremic syndrome (aHUS) is a genetic ultrarare renal disease associated
with overactivation of the alternative pathway of complement. Four gain-of-function …

Although genetic defect of complement factor H (CFH) is a common cause of atypical
hemolytic uremic syndrome (aHUS), development of autoantibodies to CFH (CFH-Ab) is …

In contrast to pregnancy-associated thrombotic thrombocytopenic purpura, the pathogenesis
and presentation of pregnancy-associated atypical hemolytic uremic syndrome (P-aHUS) …

The haemolytic uraemic syndrome (HUS) is characterized by the triad of thrombocytopenia,
microangiopathic haemolytic anaemia and acute renal failure. HUS may be classified as …