Introduction:
Defect in alternate complement pathway can cause atypical hemolytic
uremic syndrome (aHUS) which is the triad of non-immune haemolytic
anemia, thrombocytopenia and acute kidney injury (AKI). Mutation in
regulators like factor H (FH), factor I (FI), membrane cofactor protein
(MCP)/ CD46 or activators like FB or C3, remain the major underlying
pathogenesis. Further, there have been studies to suggest dual
complement abnormalities [1-4]. Moreover, children with aHUS present
with varied manifestations ranging from renal to extra-renal
manifestations like central nervous system (CNS), hepatic and cardiac
involvement. Although certain complement defects like MCP, anti-FH
antibody associated HUS, have a phenotypic relationship in clinical
manifestations and natural history, it is not applicable to most. Hence,
we tried to study the spectrum of complement abnormalities, presentation
and short term outcome in pediatric aHUS.