{"title":"与妊娠、肾移植或高血压急症有关的非典型溶血性尿毒症的最新数据","authors":"Khalil El Karoui","doi":"10.1684/ndt.2024.83","DOIUrl":null,"url":null,"abstract":"<p><p>Thrombotic microangiopathies (TMA) are a heterogeneous group of disorders characterized to varying degrees by mechanical hemolytic anemia, thrombocytopenia and histological microvascular occlusion. TMA can be complicated by multiple organ disorders, mainly neurological and nephrological (defining the hemolytic uremic syndrome, HUS). Classification based on disease pathophysiology has highlighted the importance of dysregulation of the alternative complement pathway in atypical (non-infection-related) HUS. However, the pathophysiology and treatment of many forms of aHUS in specific contexts (such as pregnancy, renal transplantation or hypertensive emergencies) remain poorly characterized. In this article, we review recent diagnostic and therapeutic advances in these three forms of aHUS in specific contexts. We show the specificities of these forms and describe the current and future challenges of their management.</p>","PeriodicalId":94153,"journal":{"name":"Nephrologie & therapeutique","volume":"20 4","pages":"277-284"},"PeriodicalIF":0.7000,"publicationDate":"2024-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":"{\"title\":\"Recent data on atypical hemolytic uremic syndrome associated with pregnancy, kidney transplantation or hypertensive emergency\",\"authors\":\"Khalil El Karoui\",\"doi\":\"10.1684/ndt.2024.83\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<p><p>Thrombotic microangiopathies (TMA) are a heterogeneous group of disorders characterized to varying degrees by mechanical hemolytic anemia, thrombocytopenia and histological microvascular occlusion. TMA can be complicated by multiple organ disorders, mainly neurological and nephrological (defining the hemolytic uremic syndrome, HUS). Classification based on disease pathophysiology has highlighted the importance of dysregulation of the alternative complement pathway in atypical (non-infection-related) HUS. However, the pathophysiology and treatment of many forms of aHUS in specific contexts (such as pregnancy, renal transplantation or hypertensive emergencies) remain poorly characterized. In this article, we review recent diagnostic and therapeutic advances in these three forms of aHUS in specific contexts. We show the specificities of these forms and describe the current and future challenges of their management.</p>\",\"PeriodicalId\":94153,\"journal\":{\"name\":\"Nephrologie & therapeutique\",\"volume\":\"20 4\",\"pages\":\"277-284\"},\"PeriodicalIF\":0.7000,\"publicationDate\":\"2024-08-01\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"Nephrologie & therapeutique\",\"FirstCategoryId\":\"1085\",\"ListUrlMain\":\"https://doi.org/10.1684/ndt.2024.83\",\"RegionNum\":0,\"RegionCategory\":null,\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"\",\"PubModel\":\"\",\"JCR\":\"\",\"JCRName\":\"\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"Nephrologie & therapeutique","FirstCategoryId":"1085","ListUrlMain":"https://doi.org/10.1684/ndt.2024.83","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"","JCRName":"","Score":null,"Total":0}
Recent data on atypical hemolytic uremic syndrome associated with pregnancy, kidney transplantation or hypertensive emergency
Thrombotic microangiopathies (TMA) are a heterogeneous group of disorders characterized to varying degrees by mechanical hemolytic anemia, thrombocytopenia and histological microvascular occlusion. TMA can be complicated by multiple organ disorders, mainly neurological and nephrological (defining the hemolytic uremic syndrome, HUS). Classification based on disease pathophysiology has highlighted the importance of dysregulation of the alternative complement pathway in atypical (non-infection-related) HUS. However, the pathophysiology and treatment of many forms of aHUS in specific contexts (such as pregnancy, renal transplantation or hypertensive emergencies) remain poorly characterized. In this article, we review recent diagnostic and therapeutic advances in these three forms of aHUS in specific contexts. We show the specificities of these forms and describe the current and future challenges of their management.