Nephromegaly in an infant with atypical hemolytic-uremic syndrome resolving successfully with eculizumab

Atypical hemolytic-uremic syndrome (aHUS) can pose a diagnostic challenge due to the multisystem involvement and varied manifestations. Early diagnosis and initiation of Eculizumab have been reported to have favorable renal outcomes. We report a case of 11-month-old male infant who presented at the age of two months with anemia, acute kidney injury, hypertension, and nephromegaly. Renal biopsy confirmed the diagnosis of aHUS and Eculizumab was started. Thrombotic microangiopathy markers showed the resolution. Kidney sizes improved after nine months of Eculizumab therapy. The successful resolution of nephromegaly in an infant with aHUS has not been reported so far in literature. Renal sizes should be monitored in all children with aHUS.