Where next with atypical hemolytic uremic syndrome?
Hemolytic uremic syndrome (HUS) is a systemic disease characterized by damage to endothelial cells, erythrocytes and kidney glomeruli. A “typical” form of HUS follows gastrointestinal infection with enterohemorrhagic E. coli (e.g. O157:H7). Atypical HUS (aHUS) is not associated with gastrointestinal infections but is sporadic or familial in nature. Approximately 50% of aHUS cases are associated with a mutation in one or more genes coding for proteins involved in regulation or activation of the alternative pathway of complement. The link between the disease and the mutations shows the important balance of the alternative pathway between activation and regulation on host cell surfaces. It also demonstrates the power of this pathway in destroying cellular targets in general. In this review we discuss the current knowledge on pathogenesis, classification, diagnostics and management of this disease. We indicate a comprehensive diagnostic approach for aHUS based on the latest knowledge on co
