论文部分内容阅读
在遗传性肾病中,称为氏综合征或芬兰人先天性肾病综合征值得注意,其病因未明。假设因母亲和胎儿免疫不适应,抗体形成并通过胎盘进入胎儿损害肾小球基底膜。最典型的组织形态为肾小管串珠样扩张——假性囊肿形成。此病常见于生后一个月内,主要症状是水肿。实验室检查与典型肾病综合征相同。有结缔组织多发性发育不良的特征。用糖类皮质醇及其他免疫抑制剂治疗无效,在一年内慢性肾功能不全发生前死于各种感染。作者曾见过5例先天性肾病综合征,3例来自同一家庭,现报导所见病例如下。
In hereditary nephropathy, it is notable that the cause of the disease is known as the syndrome or Finn’s congenital nephrotic syndrome. Assuming that the mother and fetus are not immune to immunity, antibodies form and pass through the placenta into the fetus to damage the glomerular basement membrane. The most typical form of tubular beaded tubular expansion - pseudocyst formation. The disease is common in one month after birth, the main symptom is edema. Laboratory tests are the same as typical nephrotic syndrome. Multiple connective tissue dysplasia characteristics. Corticosteroids and other immunosuppressive agents are ineffective and die of various infections before the onset of chronic renal insufficiency within one year. The authors have seen 5 cases of congenital nephrotic syndrome, 3 cases from the same family, the reported cases are reported as follows.