ArticleNagoya journal of medical science2025
Atypical hemolytic uremic syndrome treated with anti-C5 antibody agent eculizumab, without genetic complement abnormalities.
Article in Nagoya journal of medical science, 2025. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.
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Abstract
Atypical hemolytic uremic syndrome (aHUS) is a rare and life-threatening disease often complicated by end-stage renal disease. Anti-C5 antibody agents have been developed for the treatment of aHUS: these are highly effective but limited in use owing to the difficulty of diagnosing aHUS in the acute clinical phase. The pathophysiology of aHUS is a thrombotic microangiopathy (TMA) caused by complement dysregulation triggered by environmental factors in susceptible individuals with genetic factors. Although several germline variants associated with aHUS have been identified, approximately half of patients with aHUS lack known pathogenic variants. It is essential to recognize the characteristic clinical features of aHUS. These include the triad of hemolytic anemia, thrombocytopenia, and renal impairment, without the presence of Shiga toxin-producing
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