4.5 Review

New findings in preventing recurrence and improving renal function in AHUS patients after renal transplantation treated with eculizumab: a systemic review and meta-analyses

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Assessing the Impact of Prophylactic Eculizumab on Renal Graft Survival in Atypical Hemolytic Uremic Syndrome

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Summary: Prophylactic eculizumab treatment dramatically improves graft survival, making transplantation a viable therapeutic option in aHUS.

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Summary: This study retrospectively analyzed the outcome of pediatric patients with aHUS who discontinued eculizumab treatment. The study found that eculizumab can be safely discontinued with close monitoring in a selected group of patients, and if recurrence occurs, restarting eculizumab immediately can achieve complete remission.

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Efficacy and Safety of Eculizumab in Kidney Transplant Patients With Primary Atypical Hemolytic-Uremic Syndrome

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Summary: The atypical hemolytic-uremic syndrome (aHUS) is a disease characterized by non-immune hemolytic anemia, thrombocytopenia, and acute renal failure. The use of eculizumab has improved the prognosis of aHUS and prevented recurrence after kidney transplantation.

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Atypical hemolytic uremic syndrome in Brazil: clinical presentation, genetic findingss and outcomes of a case series in adults and children treated with eculizumab

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Summary: This study is the largest case series of aHUS in Brazil involving a wide range of patients for which eculizumab was the main treatment. Although eculizumab was started later than advised in the guidelines, most patients were able to stop dialysis at variable intervals. Discontinuation of eculizumab was associated with a 30% relapse of aHUS.

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Article Urology & Nephrology

Characteristics, management and outcomes of atypical haemolytic uraemic syndrome in kidney transplant patients: a retrospective national study

Jose Portoles et al.

Summary: Both pre-aHUS and de novo patients showed different clinical profiles and responses to ECU treatment. Genetic studies are important in determining risks of relapse and guiding treatment decisions. ECU may be considered as a preemptive treatment for patients at moderate or high risk of recurrence.

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The long-acting C5 inhibitor, ravulizumab, is effective and safe in pediatric patients with atypical hemolytic uremic syndrome naive to complement inhibitor treatment

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Summary: Ravulizumab rapidly improved hematologic and kidney parameters in complement inhibitor-naive children with atypical hemolytic uremic syndrome, showing no unexpected safety concerns.

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Outcome of atypical haemolytic uraemic syndrome relapse after eculizumab withdrawal

Caroline Duineveld et al.

Summary: This interim analysis evaluated the safety of re-treatment with eculizumab in 11 patients with suspected relapse. Results showed that re-treatment with eculizumab after relapse is safe. Transplanted patients responded better to eculizumab therapy compared to those with aHUS in native kidneys.

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Eculizumab and aHUS: to stop or not

Robert A. Brodsky

Summary: Evidence from a prospective study shows that discontinuing eculizumab is safe in most aHUS patients after achieving complete remission, with a relapse risk of less than 25% overall, but up to 50% in patients with rare variants in at least one complement gene.
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Kidney transplant in patients with atypical hemolytic uremic syndrome in the anti-C5 era: single-center experience with tailored Eculizumab

Gianluigi Ardissino et al.

Summary: This study presents experience with KTx in aHUS patients, showing that prophylactic use of Eculizumab can significantly reduce relapse rates. Tailored timing of Eculizumab doses based on classical complement pathway activity for each patient has also shown positive results.

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Eculizumab discontinuation in atypical haemolytic uraemic syndrome: TMA recurrence risk and renal outcomes

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Teresa Cavero et al.

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

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AMERICAN JOURNAL OF KIDNEY DISEASES (2017)

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A national specialized service in England for atypical haemolytic uraemic syndrome-the first year's experience

N. S. Sheerin et al.

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Terminal Complement Inhibitor Eculizumab in Atypical Hemolytic-Uremic Syndrome

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PEDIATRIC NEPHROLOGY (2009)

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Complement and the atypical hemolytic uremic syndrome in children

Chantal Loirat et al.

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JPT Higgins et al.

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