局灶性肾小球硬化症

0 UROLOGY & NEPHROLOGY
Varsha Suresh , Isaac E. Stillman , Kirk N. Campbell , Kristin Meliambro
{"title":"局灶性肾小球硬化症","authors":"Varsha Suresh ,&nbsp;Isaac E. Stillman ,&nbsp;Kirk N. Campbell ,&nbsp;Kristin Meliambro","doi":"10.1053/j.akdh.2024.03.009","DOIUrl":null,"url":null,"abstract":"<div><p>Focal segmental glomerular sclerosis (FSGS) is a histological lesion characterized by sclerosis in sections (segmental) of some glomeruli (focal) in association with podocyte injury. Historically, FSGS has often been characterized as a disease, but it is a heterogeneous entity based on etiology, clinical course, and therapeutic approach. A unifying feature is podocyte injury and loss, which can be primary or the result of secondary maladaptive responses to glomerular stressors. FSGS has been demonstrated over time to carry a large health burden and remains a leading glomerular cause of ESRD globally. Recent clinical practice guidelines highlight the unmet scientific need for better understanding of disease pathogenesis, particularly for immunologic etiologies, as well as more targeted therapeutic drug development. In this review, we will discuss the current FSGS classification scheme, pathophysiologic mechanisms of injury, and treatment guidelines, along with emerging and investigational therapeutics.</p></div>","PeriodicalId":72096,"journal":{"name":"Advances in kidney disease and health","volume":"31 4","pages":"Pages 275-289"},"PeriodicalIF":0.0000,"publicationDate":"2024-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":"{\"title\":\"Focal Segmental Glomerulosclerosis\",\"authors\":\"Varsha Suresh ,&nbsp;Isaac E. Stillman ,&nbsp;Kirk N. Campbell ,&nbsp;Kristin Meliambro\",\"doi\":\"10.1053/j.akdh.2024.03.009\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<div><p>Focal segmental glomerular sclerosis (FSGS) is a histological lesion characterized by sclerosis in sections (segmental) of some glomeruli (focal) in association with podocyte injury. Historically, FSGS has often been characterized as a disease, but it is a heterogeneous entity based on etiology, clinical course, and therapeutic approach. A unifying feature is podocyte injury and loss, which can be primary or the result of secondary maladaptive responses to glomerular stressors. FSGS has been demonstrated over time to carry a large health burden and remains a leading glomerular cause of ESRD globally. Recent clinical practice guidelines highlight the unmet scientific need for better understanding of disease pathogenesis, particularly for immunologic etiologies, as well as more targeted therapeutic drug development. In this review, we will discuss the current FSGS classification scheme, pathophysiologic mechanisms of injury, and treatment guidelines, along with emerging and investigational therapeutics.</p></div>\",\"PeriodicalId\":72096,\"journal\":{\"name\":\"Advances in kidney disease and health\",\"volume\":\"31 4\",\"pages\":\"Pages 275-289\"},\"PeriodicalIF\":0.0000,\"publicationDate\":\"2024-07-01\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"Advances in kidney disease and health\",\"FirstCategoryId\":\"1085\",\"ListUrlMain\":\"https://www.sciencedirect.com/science/article/pii/S2949813924000703\",\"RegionNum\":0,\"RegionCategory\":null,\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"\",\"PubModel\":\"\",\"JCR\":\"0\",\"JCRName\":\"UROLOGY & NEPHROLOGY\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"Advances in kidney disease and health","FirstCategoryId":"1085","ListUrlMain":"https://www.sciencedirect.com/science/article/pii/S2949813924000703","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"0","JCRName":"UROLOGY & NEPHROLOGY","Score":null,"Total":0}
引用次数: 0

摘要

局灶节段性肾小球硬化症(FSGS)是一种组织学病变,其特征是部分肾小球(局灶性)切片(节段性)硬化,并伴有荚膜细胞损伤。从历史上看,FSGS 通常被定性为一种疾病,但根据病因、临床过程和治疗方法,它是一种异质性实体。一个统一的特征是荚膜细胞损伤和脱失,这可能是原发性的,也可能是对肾小球应激因素的继发性适应不良反应的结果。随着时间的推移,FSGS 已被证明会带来巨大的健康负担,并且仍然是全球 ESRD 的主要肾小球病因。最近的临床实践指南强调了更好地了解疾病发病机制(尤其是免疫学病因)以及更有针对性地开发治疗药物的科学需求尚未得到满足。在本综述中,我们将讨论当前的 FSGS 分类方案、损伤的病理生理机制、治疗指南以及新兴的研究性疗法。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Focal Segmental Glomerulosclerosis

Focal segmental glomerular sclerosis (FSGS) is a histological lesion characterized by sclerosis in sections (segmental) of some glomeruli (focal) in association with podocyte injury. Historically, FSGS has often been characterized as a disease, but it is a heterogeneous entity based on etiology, clinical course, and therapeutic approach. A unifying feature is podocyte injury and loss, which can be primary or the result of secondary maladaptive responses to glomerular stressors. FSGS has been demonstrated over time to carry a large health burden and remains a leading glomerular cause of ESRD globally. Recent clinical practice guidelines highlight the unmet scientific need for better understanding of disease pathogenesis, particularly for immunologic etiologies, as well as more targeted therapeutic drug development. In this review, we will discuss the current FSGS classification scheme, pathophysiologic mechanisms of injury, and treatment guidelines, along with emerging and investigational therapeutics.

求助全文
通过发布文献求助,成功后即可免费获取论文全文。 去求助
来源期刊
CiteScore
5.30
自引率
0.00%
发文量
0
×
引用
GB/T 7714-2015
复制
MLA
复制
APA
复制
导出至
BibTeX EndNote RefMan NoteFirst NoteExpress
×
提示
您的信息不完整,为了账户安全,请先补充。
现在去补充
×
提示
您因"违规操作"
具体请查看互助需知
我知道了
×
提示
确定
请完成安全验证×
copy
已复制链接
快去分享给好友吧!
我知道了
右上角分享
点击右上角分享
0
联系我们:info@booksci.cn Book学术提供免费学术资源搜索服务,方便国内外学者检索中英文文献。致力于提供最便捷和优质的服务体验。 Copyright © 2023 布克学术 All rights reserved.
京ICP备2023020795号-1
ghs 京公网安备 11010802042870号
Book学术文献互助
Book学术文献互助群
群 号:604180095
Book学术官方微信