Daskalakis Nikki, Winn Michelle P
Department of Medicine, and the Center for Human Genetics, Duke University Medical Center, Durham, NC 27710, USA.
Semin Nephrol. 2006 Mar;26(2):89-94. doi: 10.1016/j.semnephrol.2005.09.001.
Focal and segmental glomerulosclerosis (FSGS) is a pathologic entity that is a common and increasing cause of end-stage renal disease. Typical manifestations include proteinuria, hypertension, worsening renal insufficiency, and, frequently, renal failure. The etiology, however, remains unknown in a majority of patients. There is an estimated recurrence rate of 30% to 40% in renal transplant patients, suggesting that the pathogenesis is not solely a result of intrinsic kidney disease. Although some of its characteristics have been reported, the precise identification of a circulating factor associated with FSGS has not been made. Remarkable progress has been made in recent years regarding biologic mechanisms surrounding FSGS and proteinuria. Insight into the pathogenesis of FSGS has been gained through the study of hereditary forms of FSGS and nephrotic syndromes. Mutations in cytoskeletal proteins that affect podocyte structure have been the target until recently. Here we review the current understanding of this glomerular disease and areas for future concentration.
局灶节段性肾小球硬化（FSGS）是一种病理实体，是终末期肾病常见且日益增多的病因。典型表现包括蛋白尿、高血压、肾功能不全加重，且常伴有肾衰竭。然而，大多数患者的病因仍不清楚。肾移植患者的估计复发率为30%至40%，这表明其发病机制并非仅仅是内在肾病的结果。尽管已经报道了它的一些特征，但尚未精确鉴定出与FSGS相关的循环因子。近年来，围绕FSGS和蛋白尿的生物学机制取得了显著进展。通过对遗传性FSGS和肾病综合征的研究，对FSGS的发病机制有了深入了解。直到最近，影响足细胞结构的细胞骨架蛋白突变一直是研究目标。在此，我们综述了对这种肾小球疾病的当前认识以及未来需要重点关注的领域。
