Hemolytic uremic syndrome with dual caution in an infant: cobalamin C defect and complement dysregulation successfully treated with eculizumab
2018
Background Hemolytic uremic syndrome (HUS) is a clinical syndrome characterized by
hemolytic anemia, thrombocytopenia, and
acute kidney injury.
Atypical hemolytic uremic syndrome(aHUS) is a devastating disease with significant mortality and high risk of progression to
end-stage kidney disease. It is mostly caused by dysregulation of the
alternative complement pathway.
CobalaminC (Cbl C) defect is a
genetic disorderof
cobalaminmetabolism and is a rare cause of HUS.
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