National Renal Complement Therapeutics Centre, Newcastle upon Tyne Hospitals National Health Service Foundation Trust, Newcastle upon Tyne, United Kingdom; Complement Therapeutics Research Group, Translational and Clinical Research Institute, Newcastle University, Newcastle upon Tyne, United Kingdom.
Center for Hemolytic Uremic Syndrome (HUS) Prevention, Pediatric Nephrology, Dialysis and Transplantation Unit, Fondazione Istituto di Ricovero e Cura a Carattere Scientifico (IRCCS) Ca' Granda, Ospedale Maggiore Policlinico, Milan, Italy.
Kidney Int. 2024 Dec;106(6):1038-1050. doi: 10.1016/j.kint.2024.09.012. Epub 2024 Oct 10.
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.
溶血尿毒症综合征 (HUSs) 是一组异质性疾病，其中只有部分由补体介导（补体介导的 HUS）。我们报告了 2023 年国际肾脏病学会 HUS 国际论坛的结果，该论坛全球专家组审议了目前的最新技术，确定了不确定领域，并提出了最佳解决方案。不确定领域和未来研究领域包括 HUS 的命名法、新型补体检测策略、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停药策略、肾移植受者的治疗、治疗的可及性差异，以及补体介导的 HUS 中下一代补体抑制剂。本文描述了目前最佳患者管理的基本原理。
