title: Favorable four-yr outcome after renal transplantation in a patient with complement factor H antibody and CFHR1/CFHR3 gene mutation-associated HUS.
abstract: aHUS is a clinical challenge for successful renal transplantation.
  
  
  METHODS
  A 14-yr-old girl lost her kidneys at the age of 7, due to CFH antibodies and CFH-related protein (CFHR1/CFHR3) homozygous deletion-associated aHUS. CFH, CFI, and MCP gene mutations were excluded. The patient was a candidate for renal transplantation despite persistent presence of CFH antibodies (up to 539 AU/mL). Treatment with MMF, IVIG, and repeated PF (n = 8) was introduced while being placed on urgent waiting list. Three years after aHUS onset, the patient underwent the deceased donor renal transplantation "under cover" of PF, as PF was performed directly prior to surgery and, then, PFs were repeated up to overall 14 sessions. Quadruple immunosuppression (basiliximab + tacrolimus + MMF + prednisolone) was used. Moderate symptoms of aHUS (hemolysis, low platelets, and low C3) were present within first seven days post-transplant and then normalized with PF therapy. The patient remained stable during four yr of further follow-up after transplantation.
  
  
  CONCLUSIONS
  Specific pre- and post-transplant management allowed successful renal transplantation in a CFH antibody-positive patient.
authors.affiliation: Department of Nephrology, Kidney Transplantation and Hypertension, The Children's Memorial Health Institute, Warsaw, Poland.
authors.forename: Ryszard
authors.initials: R
authors.lastname: Grenda
authors.affiliation: Department of Nephrology, Kidney Transplantation and Hypertension, The Children's Memorial Health Institute, Warsaw, Poland.
authors.forename: Wioletta
authors.initials: W
authors.lastname: Jarmużek
authors.affiliation: Department of Nephrology, Kidney Transplantation and Hypertension, The Children's Memorial Health Institute, Warsaw, Poland.
authors.forename: Jacek
authors.initials: J
authors.lastname: Rubik
authors.affiliation: Department of Nephrology, Kidney Transplantation and Hypertension, The Children's Memorial Health Institute, Warsaw, Poland.
authors.forename: Sylwester
authors.initials: S
authors.lastname: Prokurat
authors.affiliation: Department of Pediatric Nephrology, Collegium Medicum of the Jagiellonian University, Cracow, Poland.
authors.forename: Monika
authors.initials: M
authors.lastname: Miklaszewska
authors.affiliation: Department of Pediatric Nephrology, Collegium Medicum of the Jagiellonian University, Cracow, Poland.
authors.forename: Dorota
authors.initials: D
authors.lastname: Drozdz
authors.affiliation: Department of Pediatric Nephrology, Collegium Medicum of the Jagiellonian University, Cracow, Poland.
authors.forename: Katarzyna
authors.initials: K
authors.lastname: Zachwieja
authors.affiliation: Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy.
authors.forename: Gianluigi
authors.initials: G
authors.lastname: Ardissino
authors.affiliation: Department of Pediatrics, Neurology and Nephrology, Innsbruck Medical University, Innsbruck, Austria.
authors.forename: Johannes
authors.initials: J
authors.lastname: Hofer
mesh_terms: D000293:Adolescent; D065766:Atypical Hemolytic Uremic Syndrome / Q000097:blood / Q000235:genetics / Q000276:immunology / Q000601:surgery*; D001323:Autoantibodies / Q000097:blood*; D015415:Biomarkers / Q000097:blood; D001798:Blood Proteins / Q000235:genetics*; D003180:Complement C3b Inactivator Proteins / Q000235:genetics*; D017242:Complement Factor H / Q000235:genetics / Q000276:immunology*; D005260:Female; D005819:Genetic Markers; D006720:Homozygote; D006801:Humans; D016030:Kidney Transplantation; D017384:Sequence Deletion
chemical_list: D001323:Autoantibodies; D015415:Biomarkers; D001798:Blood Proteins; C517621:CFHR1 protein, human; C105970:CFHR3 protein, human; D003180:Complement C3b Inactivator Proteins; D005819:Genetic Markers; D017242:Complement Factor H
keywords: CFHR1/CFHR3 gene mutation plasmapheresis; anti-CFH Abs; atypical hemolytic uremic syndrome; renal transplantation