Niaudet P
Service de Nephrologie Pediatrique, Hôpital Necker Enfants-Malades, Paris, France.
Curr Opin Pediatr. 1993 Apr;5(2):174-9. doi: 10.1097/00008480-199304000-00007.
Proteinuria in children with idiopathic nephrotic syndrome is secondary to a loss of charge selectivity of the glomerular basement membrane. Loss of anionic charges may be secondary to a defect of heparan sulfate proteoglycans, which is also found in the congenital nephrotic syndrome, or to cationic proteins, which neutralize the anionic charges of the membrane. Reports on recurrence of nephrotic syndrome in patients with steroid-resistant idiopathic nephrotic syndrome following renal transplantation suggest that a humoral factor, possibly produced by T lymphocytes, may enhance glomerular permeability. Corticosteroids remain the basic treatment of idiopathic nephrotic syndrome. Most patients respond to steroid therapy and a high proportion of them relapse but continue to respond throughout the subsequent course of the disease. Levamisole may be effective in preventing relapses. Cyclosporine may be useful in steroid-dependent patients with signs of steroid toxicity and after a failure of a course of alkylating agent. Almost 85% of patients respond to cyclosporine, but they relapse after tapering or stopping the drug. In steroid-resistant patients, there is no study showing a clear-cut beneficial effect of alkylating agents, as the remission rate after treatment is close to the rate of spontaneous remission. Cyclosporine in association with prednisone may be effective, but the risk of nephrotoxicity seems to be higher than in steroid-dependent patients.
特发性肾病综合征患儿的蛋白尿继发于肾小球基底膜电荷选择性的丧失。阴离子电荷的丧失可能继发于硫酸乙酰肝素蛋白聚糖缺陷（先天性肾病综合征中也存在这种情况），或继发于中和膜阴离子电荷的阳离子蛋白。关于肾移植后激素抵抗型特发性肾病综合征患者肾病综合征复发的报道表明，一种可能由T淋巴细胞产生的体液因子可能会增加肾小球通透性。皮质类固醇仍然是特发性肾病综合征的基本治疗方法。大多数患者对类固醇治疗有反应，其中很大一部分会复发，但在疾病的后续过程中仍会有反应。左旋咪唑可能对预防复发有效。环孢素可能对有类固醇毒性迹象且在烷化剂疗程失败后的激素依赖型患者有用。近85%的患者对环孢素有反应，但在药物减量或停药后会复发。在激素抵抗型患者中，没有研究表明烷化剂有明确的有益效果，因为治疗后的缓解率接近自发缓解率。环孢素与泼尼松联合使用可能有效，但肾毒性风险似乎比特激素依赖型患者更高。
