Sozeri Betul, Mir Sevgi, Mutlubas Fatma, Sen Sait
Department of Pediatrics, Faculty of Medicine, Ege University, Izmir, Turkey.
Saudi J Kidney Dis Transpl. 2010 Jan;21(1):87-92.
Focal and segmental glomerulosclerosis (FSGS) is a major cause of idiopathic steroid-resistant nephrotic syndrome (SRNS) and end-stage renal disease (ESRD). In this retrospective study, we report on 34 pediatric patients with FSGS who were diagnosed and treated from 1992 to 2006. The mean age at onset was 6.3 + or - 4.3 years. All patients had nephrotic-range proteinuria. Microscopic hematuria was seen in three patients and hypertension was seen in 15 patients at presentation. All patients were treated with steroids (oral and/or methylprednisolone), while 23 patients received cytotoxic therapy in addition. The mean follow-up period was 8.6 + or - 3.3 years at the end of which, 59% of patients achieved complete or partial remission, 20.5% continued to have active renal disease while 20.5% of the patients developed CKD. Our study suggests that most of the patients with FSGS progress to renal insufficiency. Steroid therapy increases the chances of remission and preserves renal function in patients with sporadic primary FSGS.
局灶节段性肾小球硬化（FSGS）是特发性类固醇抵抗型肾病综合征（SRNS）和终末期肾病（ESRD）的主要病因。在这项回顾性研究中，我们报告了1992年至2006年期间确诊并接受治疗的34例儿童FSGS患者。发病时的平均年龄为6.3±4.3岁。所有患者均有肾病范围的蛋白尿。3例患者出现镜下血尿，15例患者就诊时出现高血压。所有患者均接受了类固醇治疗（口服和/或甲基泼尼松龙），另有23例患者还接受了细胞毒性治疗。平均随访期为8.6±3.3年，随访结束时，59%的患者实现了完全或部分缓解，20.5%的患者仍有活动性肾脏疾病，20.5%的患者发展为慢性肾脏病。我们的研究表明，大多数FSGS患者会进展为肾功能不全。类固醇治疗可增加散发性原发性FSGS患者缓解的机会并保留肾功能。
