Department of Pediatrics, Cairo University, Cairo, Egypt.
J Nephrol. 2012 Sep-Oct;25(5):732-7. doi: 10.5301/jn.5000051.
Primary nephrotic syndrome is a common renal problem in pediatrics, with great variation in patients' characteristics in different regions of the world. The aim of this study was to define these characteristics in Egyptian children with primary nephrotic syndrome.
Records of 100 primary nephrotic syndrome patients were retrospectively reviewed. Demographic, clinical, histopathological data and response to therapy were analyzed.
The mean age of onset was 4.43 ± 2.7 years. Thirty-four percent of patients were steroid resistant, and 66% showed initial steroid response; 46 of the latter were steroid dependent. Forty patients underwent a renal biopsy with minimal change nephrotic syndrome occurring in 30%, mesangioproliferative glomerulonephritis in 37.5% and focal segmental glomerulosclerosis in 30%. Nine percent of cases developed chronic renal insufficiency. Response to cyclophosphamide and cyclosporine occurred in 37.5% and 33.3% of steroid-resistant nephrotic syndrome patients, respectively.
A greater percentage of steroid-resistant patients were found in our patients compared with those in other studies. Response to immunosuppressives was different from other studies, probably due to differences in the priority of selection for immunosuppressive therapy.
原发性肾病综合征是儿科常见的肾脏问题，世界各地患者的特征存在很大差异。本研究旨在明确埃及儿童原发性肾病综合征的这些特征。
回顾性分析了 100 例原发性肾病综合征患者的病历。分析了人口统计学、临床、组织病理学数据以及对治疗的反应。
发病的平均年龄为 4.43±2.7 岁。34%的患者为激素耐药，66%的患者对激素治疗有初始反应；其中 66%的患者为激素依赖性。40 名患者进行了肾活检，其中微小病变性肾病综合征占 30%，系膜增生性肾小球肾炎占 37.5%，局灶节段性肾小球硬化症占 30%。9%的病例发展为慢性肾功能不全。对环磷酰胺和环孢素的反应分别发生在 37.5%和 33.3%的激素耐药性肾病综合征患者中。
与其他研究相比，我们的患者中发现了更高比例的激素耐药患者。对免疫抑制剂的反应与其他研究不同，这可能是由于对免疫抑制治疗的选择优先级不同所致。
