Pediatric Nephrology

, Volume 30, Issue 4, pp 603–608 | Cite as

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

  • Toshiyuki Ohta
  • Kohtaro Urayama
  • Yoshihiro Tada
  • Takeki Furue
  • Sayaka Imai
  • Keita Matsubara
  • Hiroaki Ono
  • Takashi Sakano
  • Kazuhiko Jinno
  • Yoko Yoshida
  • Toshiyuki Miyata
  • Yoshihiro Fujimura
Original Article

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.

Keywords

Plasmapheresis-resistant Meningococcal infection Prophylactic antibiotics Vaccination Pathological findings Atypical hemolytic uremic syndrome Anti-C5 therapy 

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Copyright information

© IPNA 2014

Authors and Affiliations

  • Toshiyuki Ohta
    • 1
  • Kohtaro Urayama
    • 2
  • Yoshihiro Tada
    • 3
  • Takeki Furue
    • 1
  • Sayaka Imai
    • 2
  • Keita Matsubara
    • 2
  • Hiroaki Ono
    • 2
  • Takashi Sakano
    • 2
  • Kazuhiko Jinno
    • 2
  • Yoko Yoshida
    • 4
  • Toshiyuki Miyata
    • 5
  • Yoshihiro Fujimura
    • 4
  1. 1.Department of Pediatric NephrologyHiroshima Prefectural HospitalHiroshima cityJapan
  2. 2.Department of PediatricsHiroshima Prefectural HospitalHiroshima cityJapan
  3. 3.Department of Critical CareHiroshima Prefectural HospitalHiroshima cityJapan
  4. 4.Department of Blood Transfusion MedicineNara Medical UniversityKashiharaJapan
  5. 5.Department of Molecular PathogenesisNational Cerebral and Cardiovascular CenterSuitaJapan

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