Anti-Factor H Antibody-Associated Atypical Hemolytic Uremic Syndrome: A Case Report

Abazi-Emini, Nora; Sahpazova, Emilija; Putnik, Jovana; Tasic, Velibor

doi:10.2478/prilozi-2021-0029

Cited by 2 publications

(2 citation statements)

References 14 publications

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“…The rate was not high enough to be statistically significant. The CFH produced in this group was low, and these patients were also receiving maintenance therapy, such as prednisone and tacrolimus, to prevent rejection [ 40–42 ]. In the study by Gianluigi et al.…”

Section: Discussionmentioning

confidence: 99%

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

Tang

Shi

et al. 2023

Renal Failure

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Background The long-term mortality of kidney transplantation patients with atypical hemolytic uremic syndrome remains high, and the efficacy of the main treatment eculizumab is still controversial. Objective A comprehensive systematic review and meta-analysis of clinical trials using eculizumab in renal transplant patients with atypical hemolytic uremic syndrome was conducted to evaluate the efficacy of this therapy and its impact on renal function. Methods A comprehensive systematic search was conducted across multiple reputable databases, including Ovid (MEDLINE, EMBASE), PubMed, and the Cochrane Library (since database inception), to identify relevant studies exploring the use of eculizumab in patients with atypical hemolytic uremic kidney transplantation. Various renal function parameters, such as dialysis, rejection, glomerular filtration rate, serum creatinine, lactate dehydrogenase, and platelet count, along with patient relapse rates, were extracted and summarized using a combination of robust statistical methods, including fixed effects, random effects, and general inverse variance methods. Result Eighteen trials with 618 subjects were analyzed. Our analysis suggests that the use of eculizumab is associated with a reduced likelihood of AHUS recurrence (odds ratio (OR) = 0.05, 95% CI: 0.00–0.13), as well as a significant reduction in the need for dialysis (odds ratio (OR) = 0.13, 95% CI: 0.01–0.32). Additionally, eculizumab treatment led to lower serum creatinine levels (mean differences (MD) = 126.931μmoI/L, 95% CI: 115.572μmoI/L–138.290μmoI/L) and an improved glomerular filtration rate (mean differences (MD) = 59.571 ml/min, 95% CI: 57.876 ml/min–61.266 mL/min). Our results also indicate that the use of eculizumab reduces the likelihood of rejection (odds ratio (OR) = 0.09, 95% CI: 0.01–0.22). Furthermore, the drug was effective in improving platelet counts (×10∧9/L) (mean differences (MD) = 163.421, 95% CI: 46.998–279.844) and lactate dehydrogenase levels (mean differences (MD) = 336.608 U/L, 95% CI: 164.816 U/L–508.399 U/L). Conclusions Based on the meta-analysis, treatment with eculizumab can reduce dialysis rates and improve patients’ quality of life by enhancing renal function.

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Section: Discussionmentioning

confidence: 99%

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

Tang

Shi

et al. 2023

Renal Failure

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“…It is an important etiology of acute kidney injury (AKI) and end-stage kidney disease (ESKD). Out of all typical hemolytic uremic syndrome (HUS) cases in children, 5-10% are recurrent cases of aHUS ( 1 ). Around 50% of aHUS cases arise from genetic mutations that encode regulatory proteins of the alternate complement pathway such as complement factor ( CFH ), complement factor I ( CFI ), membrane cofactor protein ( MCP ) as well as mutations in genes of complement component such as complement factor B ( CFB ) and C3.…”

Section: Introductionmentioning

confidence: 99%

Anti-factor H antibody and its role in atypical hemolytic uremic syndrome

et al. 2022

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Atypical hemolytic uremic syndrome (aHUS) an important form of a thrombotic microangiopathy (TMA) that can frequently lead to acute kidney injury (AKI). An important subset of aHUS is the anti-factor H associated aHUS. This variant of aHUS can occur due to deletion of the complement factor H genes, CFHR1 and CFHR3, along with the presence of anti-factor H antibodies. However, it is a point of interest to note that not all patients with anti-factor H associated aHUS have a CFHR1/R3 deletion. Factor-H has a vital role in the regulation of the complement system, specifically the alternate pathway. Therefore, dysregulation of the complement system can lead to inflammatory or autoimmune diseases. Patients with this disease respond well to treatment with plasma exchange therapy along with Eculizumab and immunosuppressant therapy. Anti-factor H antibody associated aHUS has a certain genetic predilection therefore there is focus on further advancements in the diagnosis and management of this disease. In this article we discuss the baseline characteristics of patients with anti-factor H associated aHUS, their triggers, various treatment modalities and future perspectives.

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Anti-Factor H Antibody-Associated Atypical Hemolytic Uremic Syndrome: A Case Report

Cited by 2 publications

References 14 publications

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

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

Anti-factor H antibody and its role in atypical hemolytic uremic syndrome

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