title: Successful living-related renal transplantation in a patient with factor H antibody-associated atypical hemolytic uremic syndrome.
abstract: CFH-Ab-associated aHUS requires different diagnostic and therapeutic approaches and then the genetically defined aHUS forms. The risk of post-transplant recurrence with graft dysfunction in CFH-Ab aHUS is not well documented. It is suggested that recurrence can be expected if a significant CFH-Ab load persists at the time of transplantation. A pretransplant procedure to reduce CFH-Ab titer seems reasonable, but accurate recommendations are lacking. Whether further prophylactic interventions after transplantation are necessary has to be decided on an individual basis. We report the case of a late diagnosed CFH-Ab HUS with initial ESRD and a successful living-related renal transplantation over a post-transplant period of four and a half years on the basis of a prophylactic pretransplant IVIG admission.
authors.affiliation: Department of Pediatrics, Innsbruck Medical University, Innsbruck, Austria.
authors.forename: Johannes
authors.initials: J
authors.lastname: Hofer
authors.affiliation: Department of Pediatrics, Innsbruck Medical University, Innsbruck, Austria.
authors.forename: Thomas
authors.initials: T
authors.lastname: Giner
authors.affiliation: Department of Pediatrics, Innsbruck Medical University, Innsbruck, Austria.
authors.forename: Gerard
authors.initials: G
authors.lastname: Cortina
authors.affiliation: Department of Pediatrics, Innsbruck Medical University, Innsbruck, Austria.
authors.forename: Therese
authors.initials: T
authors.lastname: Jungraithmayr
authors.affiliation: Clinic of Children Diseases, Hospital of Lithuanian University of Health Sciences Kaunas Clinics, Kaunas, Lithuania.
authors.forename: Jurate
authors.initials: J
authors.lastname: Masalskiene
authors.affiliation: Clinic of Children Diseases, Hospital of Lithuanian University of Health Sciences Kaunas Clinics, Kaunas, Lithuania.
authors.forename: Diana
authors.initials: D
authors.lastname: Dobiliene
authors.affiliation: Clinic of Children Diseases, Hospital of Lithuanian University of Health Sciences Kaunas Clinics, Kaunas, Lithuania.
authors.forename: Renata
authors.initials: R
authors.lastname: Mitkiene
authors.affiliation: Clinic of Children Diseases, Hospital of Lithuanian University of Health Sciences Kaunas Clinics, Kaunas, Lithuania.
authors.forename: Birute
authors.initials: B
authors.lastname: Pundziene
authors.affiliation: Clinic of Children Diseases, Hospital of Lithuanian University of Health Sciences Kaunas Clinics, Kaunas, Lithuania.
authors.forename: Sarunas
authors.initials: S
authors.lastname: Rudaitis
mesh_terms: D000906:Antibodies / Q000276:immunology*; D065766:Atypical Hemolytic Uremic Syndrome / Q000150:complications* / Q000601:surgery*; D002648:Child; D017242:Complement Factor H / Q000276:immunology*; D006085:Graft Survival; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use; D007166:Immunosuppressive Agents / Q000737:chemistry; D016030:Kidney Transplantation / Q000379:methods*; D019520:Living Donors; D008297:Male; D012008:Recurrence; D051437:Renal Insufficiency / Q000150:complications / Q000601:surgery*; D016896:Treatment Outcome
chemical_list: D000906:Antibodies; D016756:Immunoglobulins, Intravenous; D007166:Immunosuppressive Agents; D017242:Complement Factor H
keywords: atypical HUS; complement factor H antibodies; intravenous immunoglobulins; plasma therapy; renal transplantation