TY - JOUR
T1 - Favorable four-yr outcome after renal transplantation in a patient with complement factor H antibody and CFHR1/CFHR3 gene mutation-associated HUS
AU - Grenda, Ryszard
AU - Jarmuzek, Wioletta
AU - Rubik, Jacek
AU - Prokurat, Sylwester
AU - Miklaszewska, Monika
AU - Drozdz, Dorota
AU - Zachwieja, Katarzyna
AU - Ardissino, Gianluigi
AU - Hofer, Johannes
PY - 2015/9/1
Y1 - 2015/9/1
N2 - 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.
AB - 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.
KW - anti-CFH Abs
KW - atypical hemolytic uremic syndrome
KW - CFHR1/CFHR3 gene mutation plasmapheresis
KW - renal transplantation
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U2 - 10.1111/petr.12537
DO - 10.1111/petr.12537
M3 - Article
C2 - 26087050
AN - SCOPUS:84938207112
VL - 19
SP - E130-E134
JO - Pediatric Transplantation
JF - Pediatric Transplantation
SN - 1397-3142
IS - 6
ER -