title: Eculizumab and Belatacept for De Novo Atypical Hemolytic Uremic Syndrome Associated With CFHR3-CFHR1 Deletion in a Kidney Transplant Recipient: A Case Report.
abstract: BACKGROUND
  Atypical hemolytic uremic syndrome (aHUS) is associated with significant morbidity and mortality and occurs due to genetic or acquired abnormalities that result in the dysregulation of the alternative complement pathway.
  
  
  METHODS
  We report a case of post-living kidney transplantation de novo aHUS in a setting of heterozygous deletion in the complement factor H-related protein (CFHR)3-CFHR1 gene. The aHUS episode was possibly triggered by antibody-mediated rejection or tacrolimus. The patient responded well to eculizumab and substituting belatacept for tacrolimus. Her serum creatinine level was stable at 1.5 mg/dL after 2.5 years of follow-up.
  
  
  CONCLUSIONS
  This case highlights the success of using a strategy that combines eculizumab and belatacept, as an alternative to calcineurin inhibitors, in treating aHUS in a patient with heterozygous deletion in the CFHR3-CFHR1 gene.
authors.affiliation: Division of Nephrology and Hypertension, University of Cincinnati, Cincinnati, Ohio, United States. Electronic address: drparasdedhia@gmail.com.
authors.forename: P
authors.initials: P
authors.lastname: Dedhia
authors.affiliation: Division of Nephrology and Hypertension, University of Cincinnati, Cincinnati, Ohio, United States.
authors.forename: A
authors.initials: A
authors.lastname: Govil
authors.affiliation: Division of Nephrology and Hypertension, University of Cincinnati, Cincinnati, Ohio, United States.
authors.forename: G
authors.initials: G
authors.lastname: Mogilishetty
authors.affiliation: Division of Nephrology and Hypertension, University of Cincinnati, Cincinnati, Ohio, United States.
authors.forename: R R
authors.initials: RR
authors.lastname: Alloway
authors.affiliation: Division of Transplant Surgery, University of Cincinnati, Cincinnati, Ohio, United States.
authors.forename: E S
authors.initials: ES
authors.lastname: Woodle
authors.affiliation: Division of Nephrology and Hypertension, University of Cincinnati, Cincinnati, Ohio, United States.
authors.forename: B G
authors.initials: BG
authors.lastname: Abu Jawdeh
mesh_terms: D000069594:Abatacept / Q000627:therapeutic use*; D000328:Adult; D061067:Antibodies, Monoclonal, Humanized / Q000627:therapeutic use*; D065766:Atypical Hemolytic Uremic Syndrome / Q000188:drug therapy* / Q000209:etiology / Q000235:genetics; D001798:Blood Proteins / Q000235:genetics; D003180:Complement C3b Inactivator Proteins / Q000235:genetics; D051056:Complement Inactivating Agents / Q000627:therapeutic use*; D005260:Female; D006084:Graft Rejection / Q000150:complications / Q000188:drug therapy* / Q000517:prevention & control; D006579:Heterozygote; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D016030:Kidney Transplantation; D011183:Postoperative Complications / Q000188:drug therapy* / Q000235:genetics; D016559:Tacrolimus / Q000009:adverse effects
chemical_list: D061067:Antibodies, Monoclonal, Humanized; D001798:Blood Proteins; C517621:CFHR1 protein, human; C105970:CFHR3 protein, human; D003180:Complement C3b Inactivator Proteins; D051056:Complement Inactivating Agents; D007166:Immunosuppressive Agents; D000069594:Abatacept; C481642:eculizumab; D016559:Tacrolimus