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Favorable four‐yr outcome after renal transplantation in a patient with complement factor H antibody and CFHR1/CFHR3 gene mutation‐associated HUS
Authors:Ryszard Grenda  Wioletta Jarmużek  Jacek Rubik  Sylwester Prokurat  Monika Miklaszewska  Dorota Drozdz  Katarzyna Zachwieja  Gianluigi Ardissino  Johannes Hofer
Affiliation:1. Department of Nephrology, Kidney Transplantation and Hypertension, The Children's Memorial Health Institute, Warsaw, Poland;2. Department of Pediatric Nephrology, Collegium Medicum of the Jagiellonian University, Cracow, Poland;3. Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy;4. Department of Pediatrics, Neurology and Nephrology, Innsbruck Medical University, Innsbruck, Austria
Abstract:aHUS is a clinical challenge for successful renal transplantation. Case report: 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. Conclusion: Specific pre‐ and post‐transplant management allowed successful renal transplantation in a CFH antibody‐positive patient.
Keywords:atypical hemolytic uremic syndrome  anti‐CFH Abs  CFHR1/CFHR3 gene mutation plasmapheresis  renal transplantation
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