Excessive activation of the complement system in atypical hemolytic uremic syndrome: is it ready for prime time?
Complement factor I (CFI) mutations are implicated in the pathogenesis of atypical hemolytic uremic syndrome (aHUS). Nevertheless, there is evidence that CFI deficiency is a weak effector of aHUS. Bienaime et al. report that homozygous deletion of CFHR-1 in the RCA gene cluster of chromosome 1q is a major risk factor for poor outcome for patients with CFI mutations. The basic and clinical implications of the findings are further elaborated here.
Tsai
Han-Mou
HM
Division of Hematology/Oncology, Pennsylvania State University, Milton S. Hershey Medical Center, Hershey, Pennsylvania 17033, USA. htsai@hmc.psu.edu
D003167:Complement Activation; D006463:Hemolytic-Uremic Syndrome / Q000276:immunology*; D006801:Humans