Hemolytic uremic syndromes (HUSs) are a heterogeneous group of conditions, only some of which are mediated by complement (complement-mediated HUS). We report the outcome of the 2023 International Society of Nephrology HUS International Forum where a global panel of experts considered the current state of the art, identified areas of uncertainty, and proposed optimal solutions. Areas of uncertainty and areas for future research included the nomenclature of HUS, novel complement testing strategies, identification of biomarkers, genetic predisposition to atypical HUS, optimal dosing and withdrawal strategies for C5 inhibitors, treatment of kidney transplant recipients, disparity of access to treatment, and the next generation of complement inhibitors in complement-mediated HUS. The current rationale for optimal patient management is described.

中文翻译：

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国际肾脏病学会溶血性尿毒症综合征国际论坛的成果


溶血性尿毒症综合征 （HUS） 是一组异质性疾病，其中只有一部分是由补体介导的（补体介导的 HUS）。我们报告了 2023 年国际肾脏病学会 HUS 国际论坛的成果，该论坛的全球专家小组考虑了当前技术水平，确定了不确定性领域，并提出了最佳解决方案。不确定的领域和未来研究的领域包括 HUS 的命名、新的补体检测策略、生物标志物的鉴定、非典型 HUS 的遗传易感性、C5 抑制剂的最佳剂量和退出策略、肾移植受者的治疗、获得治疗的差异以及补体介导的 HUS 中的下一代补体抑制剂。描述了当前最佳患者管理的基本原理。