Safaei A, Maleknejad S
Department of Pediatrics, Guilan University of Medical Science, Rasht, IR, Iran.
Indian J Nephrol. 2009 Jul;19(3):87-90. doi: 10.4103/0971-4065.57103.
Nephrotic syndrome (NS) is a clinical entity characterized by massive loss of urinary protein (primarily albuminuria) leading to hypoproteinemia (hypoalbuminemia) and its result, edema. Hyperlipidemia, hypercholesterolemia, and increased lipiduria are usually associated. Although not commonly thought of as part of the syndrome, hypertension, hematuria, and azotemia may be present. This prospective cross-sectional study was performed on 44 children (with age of onset up to 14 years) with idiopathic nephrotic syndrome (INS) in our center during 2000-2007. The objectives were to study the clinical and biochemical parameters and the histopathological distribution of different subtypes of INS and drug-response pattern in these patients. The study group included 44 children. There were 29 (66%) boys and 15 girls (34%). The mean age was 4.87 +/- 3.24 years. Facial edema was found in 42 (95%), microscopic hematuria in 10 (23%), gross hematuria in 2 (4.5%), and hypertension in 5 (11.2%) patients. In children who underwent biopsy, focal segmental glomerulosclerosis was the most common pathologic finding (41%). Other subtypes included minimal change disease in three (18%), membranoproliferative glomerulonephritis in one (5.8%), diffuse proliferative glomerulonephritis in two (11.6 %), membranous glomerulonephritis in one (5.8%), and diffuse mesangial proliferation in three (17.5%) cases. At the time of hospitalization peritonitis was present in five (11.4%), pneumonia and upper respiratory infection (sinusitis) in eight (18%), and cellulites in two (4.5%) patients. Twenty nine patients (66%) were steroid sensitive, 9 (20.5%) steroid resistant, and 6 (13.5%) steroid dependent. Among patients with steroid-sensitive NS, 37% were nonrelapsers, 38.8% frequent relapsers, and 26.4% were infrequent relapsers. Differences seems to exist between season of incidence, suitable response to treatment with corticosteroids, and pathologic findings of biopsy in our study and other studies from Iran and other countries.
肾病综合征（NS）是一种临床病症，其特征为大量蛋白尿（主要为白蛋白尿）丢失，导致低蛋白血症（低白蛋白血症）及其后果——水肿。通常伴有高脂血症、高胆固醇血症和脂尿增加。虽然高血压、血尿和氮质血症通常不被视为该综合征的一部分，但也可能出现。本前瞻性横断面研究于2000年至2007年期间在我们中心对44名年龄在发病时不超过14岁的特发性肾病综合征（INS）儿童进行。目的是研究这些患者的临床和生化参数、INS不同亚型的组织病理学分布以及药物反应模式。研究组包括44名儿童。其中男孩29名（66%），女孩15名（34%）。平均年龄为4.87±3.24岁。42名（95%）患者出现面部水肿，10名（23%）出现镜下血尿，2名（4.5%）出现肉眼血尿，5名（11.2%）患者出现高血压。在接受活检的儿童中，局灶节段性肾小球硬化是最常见的病理表现（41%）。其他亚型包括3名（18%）微小病变病、1名（5.8%）膜增生性肾小球肾炎、2名（11.6%）弥漫性增生性肾小球肾炎、1名（5.8%）膜性肾小球肾炎以及3名（17.5%）弥漫性系膜增生。住院时，5名（11.4%）患者发生腹膜炎，8名（18%）患者发生肺炎和上呼吸道感染（鼻窦炎），2名（4.5%）患者发生蜂窝织炎。29名患者（66%）对类固醇敏感，9名（20.5%）对类固醇耐药，6名（13.5%）对类固醇依赖。在对类固醇敏感的NS患者中，37%为非复发者，38.8%为频繁复发者，26.4%为不频繁复发者。在我们的研究与伊朗及其他国家的其他研究之间，发病率季节、对皮质类固醇治疗的适当反应以及活检的病理结果似乎存在差异。
