title: Eculizumab as rescue therapy for atypical hemolytic uremic syndrome with normal platelet count.
abstract: BACKGROUND
  Atypical hemolytic uremic syndrome (aHUS) in childhood is a rare disease with frequent progression to end-stage renal disease and a high recurrence after kidney transplantation. Eculizumab, a humanized monoclonal antibody that binds to complement protein C5, may be beneficial in the treatment of aHUS.
  
  
  METHODS
  A 6-year-old girl developed aHUS with only slightly elevated C3d (4.4%), no mutations in complement factors, and no antibodies against factor H. Plasma exchange treatment was successful initially, until aHUS recurred. After reinitiating plasma exchange, normalization of the platelet count and improvement of hemolysis occurred, but renal function worsened. Renal function then improved dramatically promptly after the switch to eculizumab.
  
  
  CONCLUSIONS
  This case demonstrates that platelet count is not always a reliable marker for improvement of aHUS and that eculizumab can prevent dialysis in plasma-resistant aHUS patients.
authors.affiliation: Pediatric Nephrology, Erasmus MC - Sophia Childrens Hospital, Dr Molewaterplein 60, 3015 GJ, Rotterdam, The Netherlands. e.dorresteijn@erasmusmc.nl
authors.forename: Eiske M
authors.initials: EM
authors.lastname: Dorresteijn
authors.forename: Nicole C A J
authors.initials: NC
authors.lastname: van de Kar
authors.forename: Karlien
authors.initials: K
authors.lastname: Cransberg
mesh_terms: D058186:Acute Kidney Injury / Q000188:drug therapy* / Q000209:etiology; D061067:Antibodies, Monoclonal, Humanized / Q000627:therapeutic use*; D065766:Atypical Hemolytic Uremic Syndrome; D002648:Child; D005260:Female; D006463:Hemolytic-Uremic Syndrome / Q000097:blood* / Q000150:complications / Q000188:drug therapy*; D006801:Humans; D010976:Platelet Count; D016879:Salvage Therapy / Q000379:methods*
chemical_list: D061067:Antibodies, Monoclonal, Humanized; C481642:eculizumab