Favorable four-yr outcome after renal transplantation in a patient with complement factor H antibody and CFHR1/CFHR3 gene mutation-associated HUS

Ryszard Grenda, Wioletta Jarmuzek, Jacek Rubik, Sylwester Prokurat, Monika Miklaszewska, Dorota Drozdz, Katarzyna Zachwieja, Gianluigi Ardissino, Johannes Hofer

Research output: Contribution to journalArticlepeer-review

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.

Original languageEnglish
Pages (from-to)E130-E134
JournalPediatric Transplantation
Volume19
Issue number6
DOIs
Publication statusPublished - Sep 1 2015

Keywords

  • anti-CFH Abs
  • atypical hemolytic uremic syndrome
  • CFHR1/CFHR3 gene mutation plasmapheresis
  • renal transplantation

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Transplantation

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