Eculizumab in the treatment of atypical hemolytic uremic syndrome in an infant leads to cessation of peritoneal dialysis and improvement of severe hypertension

Background Severe hypertension (HTN) and acute kidney injury frequently associated with atypical hemolytic uremic syndrome (aHUS) were refractory to various therapies in the pre-eculizumab era. Here we report the case of a 4-month-old boy who developed aHUS presenting with undetectable C3 protein, no predisposing mutations in complement factors, and no antibodies against factor H. Methods Repeated plasma infusions and nine sessions of plasmapheresis were ineffective. The patient initially required continuous hemodiafiltration and thereafter peritoneal dialysis. Despite vigorous antihypertensive treatment and improved fluid overload with dialysis, HTN persisted. His low C3 level ( Conclusions This infant with HTN and acute kidney injury associated with aHUS was treated successfully with eculizumab. Keywords Plasmapheresis-resistant * Meningococcal infection * Prophylactic antibiotics * Vaccination * Pathological findings * Atypical hemolytic uremic syndrome * Anti-C5 therapy
Introduction Hemolytic uremic syndrome (HUS) is a rare disease with manifestations of microangiopathic hemolytic anemia, thrombocytopenia, and renal impairment. There are two types of HUS: HUS which follows a diarrheal [...]