title: Eculizumab in the treatment of atypical hemolytic uremic syndrome in an infant leads to cessation of peritoneal dialysis and improvement of severe hypertension.
abstract: 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 (<20 mg/dl) suggested unrestricted complement activation. Therefore, based on the suspicion of unrestricted complement cascade in the pathogenesis, treatment with eculizumab, a human anti-C5 monoclonal antibody, was initiated with the aim of controlling disease activity.
  
  
  RESULTS
  Eculizumab therapy resulted in the control of severe HTN and cessation of peritoneal dialysis.
  
  
  CONCLUSIONS
  This infant with HTN and acute kidney injury associated with aHUS was treated successfully with eculizumab.
authors.affiliation: Department of Pediatric Nephrology, Hiroshima Prefectural Hospital, 1-5-54 Ujina-Kanda, Minami-ku, Hiroshima city, Hiroshima, 734-8530, Japan, t-ohta@hph.pref.hiroshima.jp.
authors.forename: Toshiyuki
authors.initials: T
authors.lastname: Ohta
authors.forename: Kohtaro
authors.initials: K
authors.lastname: Urayama
authors.forename: Yoshihiro
authors.initials: Y
authors.lastname: Tada
authors.forename: Takeki
authors.initials: T
authors.lastname: Furue
authors.forename: Sayaka
authors.initials: S
authors.lastname: Imai
authors.forename: Keita
authors.initials: K
authors.lastname: Matsubara
authors.forename: Hiroaki
authors.initials: H
authors.lastname: Ono
authors.forename: Takashi
authors.initials: T
authors.lastname: Sakano
authors.forename: Kazuhiko
authors.initials: K
authors.lastname: Jinno
authors.forename: Yoko
authors.initials: Y
authors.lastname: Yoshida
authors.forename: Toshiyuki
authors.initials: T
authors.lastname: Miyata
authors.forename: Yoshihiro
authors.initials: Y
authors.lastname: Fujimura
mesh_terms: D061067:Antibodies, Monoclonal, Humanized / Q000627:therapeutic use*; D065766:Atypical Hemolytic Uremic Syndrome / Q000188:drug therapy* / Q000503:physiopathology; D051056:Complement Inactivating Agents / Q000627:therapeutic use*; D006801:Humans; D006978:Hypertension, Renovascular / Q000503:physiopathology*; D007223:Infant; D008297:Male; D010530:Peritoneal Dialysis; D010956:Plasmapheresis
chemical_list: D061067:Antibodies, Monoclonal, Humanized; D051056:Complement Inactivating Agents; C481642:eculizumab