Davin J C, Mahieu P R, Dechenne C
Nephron. 1985;39(1):59-60. doi: 10.1159/000183339.
A boy presenting with a severe congenital nephrotic syndrome diagnosed by histological analysis at the age of 3 weeks was biopsied again 7 years later. The ultrastructural glomerular basement membrane abnormalities depicted in the first biopsy were no longer present in the second one. The number of completely hyalinized glomeruli was not significantly decreased. The GFR remained normal, but a moderate persistent, non-selective proteinuria (800 mg/24 h) was noted without oedema. The patient however developed a progressive perceptive deficit of hearing.
一名3周大时经组织学分析诊断为严重先天性肾病综合征的男孩，7年后再次接受活检。第一次活检中描述的肾小球基底膜超微结构异常在第二次活检中已不复存在。完全玻璃样变的肾小球数量没有显著减少。肾小球滤过率保持正常，但出现了中度持续性非选择性蛋白尿（800mg/24小时），且无水肿。然而，该患者出现了进行性听力感知缺陷。
