title: Prophylactic Eculizumab Use in Kidney Transplantation: A Review of the Literature and Report of a Case with Atypical Hemolytic Uremic Syndrome.
abstract: BACKGROUND
  Atypical hemolytic uremic syndrome (aHUS) is a very rare disease, which presents with microangiopathic hemolytic anemia, thrombocytopenia, and acute kidney injury. Progression to end-stage renal disease (ESRD) from acute kidney injury is observed in 60% of aHUS cases. The prognosis of aHUS patients who undergo kidney transplantation (Ktx) is generally poor, but these patients should be treated prophylactically with eculizumab to prevent recurrence after transplantation.
  
  
  METHODS
  An 18-year-old man was referred to our center with a history of rapid progression to ESRD with unknown etiology. He had anemia, thrombocytopenia, high levels of LDH, and indirect bilirubin and creatinine on initial laboratory results. Our diagnosis was aHUS due to initial results, normal level of ADAMTS activity, and lack of predisposing factors seen in typical HUS. We planned to perform genetic analysis for the patient and the donor candidate (mother). The variations found on exon 7 of the CFH gene had not been reported previously. According to PolyPhen analysis, this mutation was reported as a potential cause for aHUS. We decided to perform Ktx under eculizumab prophylaxis. Weekly administration of prophylaxis was extended to 1 month. The graft functioned immediately after Ktx. The patient has completed his first year uneventfully in our follow-up, with a creatinine 0.79 mg/dl at his last control visit.
  
  
  CONCLUSIONS
  We found favorable results of an aHUS case successfully treated with kidney transplantation combined with short-term prophylactic eculizumab therapy.
authors.affiliation: Department of Nephrology, Haydarpasa Numune Education and Research Hospital, İstanbul, Turkey.
authors.forename: Umut
authors.initials: U
authors.lastname: Kasapoğlu
authors.affiliation: Department of Nephrology, Haydarpasa Numune Education and Research Hospital, İstanbul, Turkey.
authors.forename: Çaglar
authors.initials: Ç
authors.lastname: Ruhi
authors.affiliation: Department of Nephrology, Haydarpasa Numune Education and Research Hospital, İstanbul, Turkey.
authors.forename: Murat
authors.initials: M
authors.lastname: Tuğcu
authors.affiliation: Department of Nephrology, Haydarpasa Numune Education and Research Hospital, İstanbul, Turkey.
authors.forename: Başak
authors.initials: B
authors.lastname: Boynueğri
authors.affiliation: Department of General Surgery and Transplantation Unit, Haydarpasa Numune Education and Research Hospital, İstanbul, Turkey.
authors.forename: İzzet
authors.initials: İ
authors.lastname: Titiz
authors.affiliation: Department of Molecular Biology and Genetics, Faculty of Science, Istanbul Bilim University, İstanbul, Turkey.
authors.forename: Veysel Sabri
authors.initials: VS
authors.lastname: Hançer
authors.affiliation: Department of Nephrology, Haydarpasa Numune Education and Research Hospital, İstanbul, Turkey.
authors.forename: Süheyla
authors.initials: S
authors.lastname: Apaydın
mesh_terms: D000293:Adolescent; D061067:Antibodies, Monoclonal, Humanized / Q000008:administration & dosage*; D065766:Atypical Hemolytic Uremic Syndrome / Q000150:complications* / Q000175:diagnosis; D018450:Disease Progression; D005500:Follow-Up Studies; D006084:Graft Rejection / Q000517:prevention & control; D006085:Graft Survival; D006801:Humans; D007279:Injections, Subcutaneous; D007676:Kidney Failure, Chronic / Q000209:etiology / Q000503:physiopathology / Q000601:surgery*; D016030:Kidney Transplantation / Q000379:methods*; D008297:Male; D011300:Preoperative Care / Q000379:methods; D011322:Primary Prevention / Q000379:methods; D018570:Risk Assessment; D013997:Time Factors; D016896:Treatment Outcome; D014421:Turkey
chemical_list: D061067:Antibodies, Monoclonal, Humanized; C481642:eculizumab