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Favorable four‐yr outcome after renal transplantation in a patient with complement factor H antibody and CFHR 1/ CFHR 3 gene mutation‐associated HUS
Author(s) -
Grenda Ryszard,
Jarmużek Wioletta,
Rubik Jacek,
Prokurat Sylwester,
Miklaszewska Monika,
Drozdz Dorota,
Zachwieja Katarzyna,
Ardissino Gianluigi,
Hofer Johannes
Publication year - 2015
Publication title -
pediatric transplantation
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.457
H-Index - 69
eISSN - 1399-3046
pISSN - 1397-3142
DOI - 10.1111/petr.12537
Subject(s) - medicine , basiliximab , transplantation , eculizumab , immunosuppression , factor h , tacrolimus , kidney transplantation , atypical hemolytic uremic syndrome , gastroenterology , immunology , antibody , complement system
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 ( CFHR 1/ CFHR 3) 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, PF s 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.