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- Title
Early relapse of atypical hemolytic uremic syndrome following ABO-incompatible living–related pediatric kidney re-transplant successfully treated with eculizumab.
- Authors
Stojanovic, Jelena; Adamusiak, Anna; Waters, Aoife; Sebire, Neil J.; Kessaris, Nicos; Mamode, Nizam; Marks, Stephen D.
- Abstract
Background: A 3-year-old girl with clinical features of atypical HUS (complement Factor I mutation inherited from an asymptomatic mother and Factor H autoantibodies) was treated with plasma exchange, progressed to kidney failure (KF) aged 4 years, and received an en bloc kidney DCD transplant aged 8 years with primary graft non-function necessitating transplant nephrectomy at the time of transplantation. She subsequently underwent re-transplantation from her father. This is a retrospective study of electronic patient records and medical notes. Case–Diagnosis/Treatment: A 9-year-old girl received an ABO-incompatible (ABOi) living–related kidney transplant from her father with recipient and donor blood groups of O and A, respectively, with baseline recipient anti-A titers 1:128 reducing to 1:4 at the time of transplant with B lymphocyte depletion with rituximab and four sessions of immunoadsorption. Six hours post-transplant, she had recurrence of aHUS and received the first dose of eculizumab. She continues on monthly home eculizumab infusions with stable kidney allograft function and negative anti-A titers 7 years post-kidney transplantation. Conclusions: This is the first report of a pediatric high-risk ABOi living–related kidney transplantation in whom early relapse of aHUS was successfully treated with eculizumab with good long-term patient and allograft outcome.
- Subjects
THERAPEUTIC use of monoclonal antibodies; ABO blood group system; RITUXIMAB; HOMOGRAFTS; KIDNEY transplantation; IMMUNOADSORPTION; DISEASE relapse; HEMOLYTIC-uremic syndrome; BLOOD group incompatibility; TRANSPLANTATION of organs, tissues, etc.; LYMPHOCYTE count; CHILDREN
- Publication
Pediatric Nephrology, 2021, Vol 36, Issue 10, p3271
- ISSN
0931-041X
- Publication type
Article
- DOI
10.1007/s00467-021-05193-7