Abstract
Context: To date, several studies have been done regarding the treatment of atypical hemolytic uremic syndrome (aHUS) which discussed eculizumab as a potential treatment for this syndrome. However, the safety and efficacy of eculizumab were not fully assessed. This study aims to do a systematic review about the efficacy and safety of eculizumab in treatment of aHUS. Evidence Acquisitions: An electronic literature search was conducted to identify appropriate studies. We included all randomized trials and observational studies about using eculizumab in aHUS. Two independent reviewers extracted data from the articles according to the selection criteria. Results: Eligible studies were included in this systematic review. The literature search and reference mining yielded 571 potential relevant articles. We removed 173 articles because of duplication. We also excluded 245 articles after reviewing the titles and abstracts, and removed 61 studies because the topics were not relevant to the subject. Finally, five studies were included in the systematic review. Conclusions: Acknowledging the limitations of the study due to the size and nature of the included studies, our systematic review shows that eculizumab was effective in the treatment of aHUS. However, further large randomized trials are suggested.
Highlights
This study aims to do a systematic review about the efficacy and safety of eculizumab in treatment of Atypical hemolytic uremic syndrome (aHUS)
Atypical hemolytic uremic syndrome is a rare disease, which is usually characterized by acute kidney injury (AKI), thrombocytopenia and microangiopathic hemolytic anemia (MAHA) (2)
Molecular and serological investigations, aHUS can be differentiated from typical HUS (Shiga toxin-producing Escherichia coli), which is related to a preceding enterohemorrhagic E. coli infection (EHEC) (1,2)
Summary
Atypical hemolytic uremic syndrome (aHUS) is a rare disease, which is usually characterized by acute kidney injury (AKI), thrombocytopenia and microangiopathic hemolytic anemia (MAHA) (2). The atypical form of aHUS has a poor prognosis where up to 50% of cases may result in end-stage renal disease, and up to 25% of lethal outcomes progress to an acute phase (3-5). Complement dysregulation which results in glomerular endothelial cell damage is known to be a significant element in aHUS etiology (3-5). TMA may result in acute renal dysfunction in early phases of the disease (6,7). Eculizumab (Soliris) is a monoclonal antibody which binds to C5 and prevents its division into C5a and C5b. It fully blocks the formation of terminal complement complex (C5b-9) (5).
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