Atypical hemolytic uremic syndrome (aHUS) is a severe renal disease that is associated with
defective complement regulation caused by multiple factors. We previously described the …

Factor H autoantibodies have been reported in approximately 10% of patients with atypical
hemolytic uremic syndrome (aHUS) and are associated with deficiency of factor H–related …

The factor H–related protein family (CFHR) is a group of minor plasma proteins genetically
and structurally related to complement factor H (fH). Notably, deficiency of CFHR1/CFHR3 …

Haemolytic uraemic syndrome (HUS) is a severe disease with renal failure,
microangiopathic anemia and thrombocytopenia. Several mechanisms leading to HUS have …

Results Homozygous deletion in CFHR1 was detected in 32% of the patients with aHUS
tested, compared with 2.5% of controls (P< 0.001). CFH antibodies were present in 25% of …

The atypical form of the kidney disease hemolytic uremic syndrome (aHUS) is associated
with defective complement regulation. In addition to mutations in complement regulators …

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 …

Atypical hemolytic uremic syndrome (aHUS) is associated with defective complement
regulation. Disease-associated mutations have been described in the genes encoding the …

Background Mutations in complement factor H (CFH), factor I (CFI), factor B (CFB),
thrombomodulin (THBD), C3 and membrane cofactor protein (MCP), and autoantibodies …

Central to the pathogenesis of atypical hemolytic uremic syndrome (aHUS) is over-activation
of the alternative pathway of complement. Following the initial discovery of mutations in the …