局灶节段性肾小球硬化症（FSGS）是儿童常见肾小球疾病，临床多表现为肾病综合征。糖皮质激素是FSGS 的一线治疗药物，但相当一部分患儿对激素治疗反应不佳，最终进展至终末期肾病，因此各种新型FSGS治疗药物不断开 发，并取得一定疗效。文章综述儿童原发性FSGS的治疗进展。

Focal segmental glomerular sclerosis (FSGS) is a common glomerular disease in children. Its clinical manifestations are mostly nephrotic syndrome. Some children do not respond well to glucocorticoids treatment, the first line treatment, and eventually progress to end-stage renal disease. Therefore, a variety of new drugs for the treatment of FSGS have been gradually developed and achieved certain efficacy. This paper reviews the treatment progress of primary FSGS in children in order to provide the basis and ideas for treatment and scientific research