Living Donor Kidney Transplantation in Atypical Hemolytic Uremic Syndrome:A Case Series

Background: The development of complement inhibitors has greatly improved the outcome of patients with atypical hemolytic uremic syndrome (aHUS), making kidney transplantation a more feasible option. Although prophylactic eculizumab therapy may prevent recurrent disease after transplantation, its necessity for all transplant recipients is debated. Study Design: A case series. Setting & Participants: Patients with aHUS who underwent living donor kidney transplantation after 2011 at 2 university centers, prospectively followed up with a protocol of eculizumab therapy limited to only recipien... Mehr ...

Verfasser: Duineveld, Caroline
Verhave, Jacobien C
Berger, Stefan P
van de Kar, Nicole C A J
Wetzels, Jack F M
Dokumenttyp: Artikel
Erscheinungsdatum: 2017
Reihe/Periodikum: Duineveld , C , Verhave , J C , Berger , S P , van de Kar , N C A J & Wetzels , J F M 2017 , ' Living Donor Kidney Transplantation in Atypical Hemolytic Uremic Syndrome : A Case Series ' , American Journal of Kidney Diseases , vol. 70 , no. 6 , pp. 770-777 . https://doi.org/10.1053/j.ajkd.2017.06.024
Schlagwörter: Acute Kidney Injury / Adult / Aged / Antibodies / Monoclonal / Humanized / Atypical Hemolytic Uremic Syndrome / BK Virus / Complement C3 / Complement Factor H / Complement Inactivating Agents / Female / Follow-Up Studies / Graft Rejection / Humans / Immunosuppressive Agents / Kidney Transplantation / Living Donors / Male / Membrane Cofactor Protein / Middle Aged / Mutation / Netherlands / Polyomavirus Infections / Postoperative Complications / Recurrence / Retrospective Studies / Tumor Virus Infections / Young Adult / Journal Article
Sprache: Englisch
Permalink: https://search.fid-benelux.de/Record/base-29192228
Datenquelle: BASE; Originalkatalog
Powered By: BASE
Link(s) : https://hdl.handle.net/11370/ba8f7904-c167-4327-8cab-6666ead19834

Background: The development of complement inhibitors has greatly improved the outcome of patients with atypical hemolytic uremic syndrome (aHUS), making kidney transplantation a more feasible option. Although prophylactic eculizumab therapy may prevent recurrent disease after transplantation, its necessity for all transplant recipients is debated. Study Design: A case series. Setting & Participants: Patients with aHUS who underwent living donor kidney transplantation after 2011 at 2 university centers, prospectively followed up with a protocol of eculizumab therapy limited to only recipients with documented posttransplantation recurrent thrombotic microangiopathy. In addition, the protocol emphasized lower target level tacrolimus and aggressive treatment of high blood pressure. Outcomes: Recurrence of aHUS, kidney function, acute kidney injury. Results: We describe 12 female and 5 male patients with a mean age of 47 years. 5 patients had lost a previous transplant due to aHUS recurrence. 16 patients carried a pathogenic or likely pathogenic variant in genes encoding complement factor H, C3, or membrane cofactor protein, giving a high risk for aHUS recurrence. Median follow-up after transplantation was 25 (range, 7-68) months. One patient had aHUS recurrence 68 days after transplantation, which was successfully treated with eculizumab. 3 patients were treated for rejection and 2 patients developed BK nephropathy. At the end of follow-up, median serum creatinine concentration was 106 (range, 67-175) mmol/L and proteinuria was negligible. Limitations: Small series and short duration of follow-up. Conclusions: Living donor kidney transplantation in aHUS without prophylactic eculizumab treatment appears feasible.