Watanabe Y, Ozaki I, Yoshida F, Fukatsu A, Itoh Y, Matsuo S, Sakamoto N
Third Department of Internal Medicine, Nagoya University School of Medicine, Japan.
Nephron. 1989;51(2):265-70. doi: 10.1159/000185297.
A 33-year-old man with nephrotic syndrome and rapid deterioration of renal function showed curious glomerular morphological abnormalities. Ballooning of the glomerular capillaries due to a substance accumulated in the capillary lumina and mesangiolysis were prominent histological features. The deposits in the capillary lumen were positive for Sudan III staining, and also for beta-lipoprotein, apoprotein B and apoprotein E by immunofluorescent technique. The staining of beta-lipoprotein in a flower leaf pattern was a striking characteristic, while such staining was negative when studied in 20 patients with nephrotic syndrome who were used as controls. Based on these findings, the morphological abnormalities in this case were considered to be related to lipoprotein deposition in the glomeruli. This case is thought to be the first reported in a complete form in the literature which could be classified as a new kind of disease related to lipoprotein metabolism abnormalities.
一名患有肾病综合征且肾功能迅速恶化的33岁男性表现出奇特的肾小球形态异常。肾小球毛细血管腔内积聚物质导致毛细血管气球样变以及系膜溶解是突出的组织学特征。毛细血管腔内的沉积物苏丹III染色呈阳性，免疫荧光技术检测显示其β-脂蛋白、载脂蛋白B和载脂蛋白E也呈阳性。β-脂蛋白呈花叶状染色是一个显著特征，而在作为对照的20例肾病综合征患者中进行研究时，这种染色呈阴性。基于这些发现，该病例的形态学异常被认为与肾小球内脂蛋白沉积有关。该病例被认为是文献中首例完整报道的可归类为一种新型与脂蛋白代谢异常相关疾病的病例。
