Sreedharan Rajasree, Bockenhauer Detlef
Department of Pediatrics, Division of Nephrology, Yale University School of Medicine, Yale University, New Haven, Connecticut, USA.
Pediatr Nephrol. 2005 Sep;20(9):1340-2. doi: 10.1007/s00467-005-1918-5. Epub 2005 Jun 18.
Congenital nephrotic syndrome is a severe disorder caused by increased permeability of the glomerular capillary leading to massive proteinuria. Typically, this disorder presents in the first three months and is caused by inherited mutations in genes encoding structural proteins of the podocyte slit membrane and, as such, is usually irreversible. Medical management is often insufficient to stem the enormous losses of protein, and the patients require nephrectomies. Here, we present results for a patient with congenital nephrotic syndrome of unknown etiology which responded to treatment with an angiotensin-converting enzyme inhibitor alone. The patient's proteinuria relapsed when the medication was stopped, but went into complete remission after restarting treatment. This remarkable response is discussed in the light of recent investigations into the effect of angiotensin II on podocyte integrity.
先天性肾病综合征是一种由肾小球毛细血管通透性增加导致大量蛋白尿的严重病症。通常，这种病症在出生后的前三个月出现，是由编码足细胞裂孔膜结构蛋白的基因发生遗传突变引起的，因此通常是不可逆的。药物治疗往往不足以阻止大量蛋白质流失，患者需要进行肾切除术。在此，我们报告了一名病因不明的先天性肾病综合征患者单独使用血管紧张素转换酶抑制剂治疗取得的效果。停药后患者蛋白尿复发，但重新开始治疗后完全缓解。根据最近关于血管紧张素II对足细胞完整性影响的研究，对这一显著反应进行了讨论。
