摘要
非典型溶血尿毒综合征(aHUS),属于血栓性微血管病,是表现为微血管病性溶血性贫血、血小板减少和急性肾损伤的临床综合征。病情常危重,未经有效治疗常致死亡或肾衰竭。文章将论述aHUS的发病机制、精准诊疗的最新进展,并展望可能的研究方向。
Atypical hemolytic uremic syndrome(aHUS)is a major thrombotic microangiopathy,which manifests as microangiopathic hemolytic anemia,thrombocytopenia,and acute kidney injury.The disease is usually critical and may cause death or renal failure if there is no effective treatment.With the usage of complement inhibitors,the prognosis of aHUS improved profoundly recent years.This review discussesthe pathogenesis of a HUS,and the latest progress of the precision diagnosis and treatment of the disease,and makes a prospect of future direction of researches.
作者
乐偲
陈丽萌
YUE Cai;CHEN Li-meng(Department of Nephrology,State Key Laboratory of Complex Severe and Rare Diseases,Peking Union Medical College Hospital,Chinese Academy of Medical Science and Peking Union Medical College,Beijing 100730,China)
出处
《中国实用内科杂志》
CAS
CSCD
北大核心
2023年第3期188-192,共5页
Chinese Journal of Practical Internal Medicine
基金
国家重点研发计划“生育健康及妇女儿童健康保障”专项(2022YFC2703901)
科技创新2030-“新一代人工智能(2030)”重大项目(2022ZD0116003)
中央高水平医院临床科研业务费(2022-PUMCH-A-171,2022-PUMCH-B-019,2022-PUMCH-B-020)
中国医学科学院医学与健康科技创新工程项目(CIFMS 2021-I2M-1-003)
北京市自然科学基金-海淀原始创新联合基金(L202035)
首都卫生发展科研专项(CFH 2020-2-4018)。
关键词
非典型溶血尿毒综合征
补体系统
补体抑制剂
atypical hemolytic uremic syndrome
complement system
complement inhibitors
