Wang Chia-Shi, Greenbaum Larry A
Division of Pediatric Nephrology, Department of Pediatrics, Emory University School of Medicine and Children's Healthcare of Atlanta, 2015 Uppergate Drive Northeast, Atlanta, GA 30322-1015, USA.
Division of Pediatric Nephrology, Department of Pediatrics, Emory University School of Medicine and Children's Healthcare of Atlanta, 2015 Uppergate Drive Northeast, Atlanta, GA 30322-1015, USA.
Pediatr Clin North Am. 2019 Feb;66(1):73-85. doi: 10.1016/j.pcl.2018.08.006.
Nephrotic syndrome is characterized by edema, proteinuria, hypoalbuminemia, and hyperlipidemia. Minimal change disease, the most common cause in childhood, generally responds to corticosteroids, although most patients experience disease relapses. Focal segmental glomerulosclerosis is usually resistant to corticosteroids and carries a significant risk of kidney failure, necessitating renal transplantation. Nephrotic syndrome may also be secondary to gene mutations and systemic diseases such as lupus. Clinical evaluation involves distinguishing primary and secondary causes and monitoring for disease complications, including blood clots and serious infections such as spontaneous bacterial peritonitis. Immunosuppressive medications are used to prevent relapses and treat corticosteroid-resistant disease.
肾病综合征的特征是水肿、蛋白尿、低白蛋白血症和高脂血症。微小病变病是儿童最常见的病因，通常对皮质类固醇有反应，不过大多数患者会经历疾病复发。局灶节段性肾小球硬化通常对皮质类固醇耐药，并伴有显著的肾衰竭风险，需要进行肾移植。肾病综合征也可能继发于基因突变和系统性疾病，如狼疮。临床评估包括区分原发性和继发性病因，并监测疾病并发症，包括血栓和严重感染，如自发性细菌性腹膜炎。免疫抑制药物用于预防复发和治疗皮质类固醇耐药性疾病。
