Obara K, Saito T, Sato H, Ogawa M, Igarashi Y, Yoshinaga K
Second Department of Internal Medicine, Tohoku University School of Medicine, Sendai, Japan.
Clin Nephrol. 1993 Feb;39(2):59-64.
Two adult patients with type I glycogen storage disease (I-GSD) had chronic renal disease with heavy proteinuria. Renal biopsies showed focal glomerular sclerosis, interstitial fibrosis, tubular atrophy or vacuolation, and prominent arteriosclerosis. Marked glomerular hypertrophy was demonstrated histometrically. Oil red O staining in one patient revealed numerous lipid deposits in the glomerular mesangium, tubular epithelial cells and interstitium. Electron microscopy in the other patient revealed diffuse thickening of the glomerular basement membrane (GBM) and lipid droplets within the mesangium. The glomerular hypertrophy, thickening of the GBM, and subsequent sclerosis were similar to those in insulin-dependent diabetes mellitus. These findings may explain the similarities between the natural histories of renal involvement in the two disorders. Particularly, glomerular hypertrophy may be a key step leading to glomerular sclerosis, which is the predominant finding I-GSD. Hyperlipidemia, which is commonly seen in I-GSD, may also accelerate the glomerular sclerosing process.
两名患有I型糖原贮积病（I-GSD）的成年患者患有慢性肾病并伴有大量蛋白尿。肾活检显示局灶性肾小球硬化、间质纤维化、肾小管萎缩或空泡形成以及显著的动脉硬化。组织测量学显示有明显的肾小球肥大。一名患者的油红O染色显示肾小球系膜、肾小管上皮细胞和间质中有大量脂质沉积。另一名患者的电子显微镜检查显示肾小球基底膜（GBM）弥漫性增厚以及系膜内有脂质滴。肾小球肥大、GBM增厚以及随后的硬化与胰岛素依赖型糖尿病中的情况相似。这些发现可能解释了这两种疾病肾脏受累自然病程之间的相似性。特别是，肾小球肥大可能是导致肾小球硬化的关键步骤，而肾小球硬化是I-GSD的主要表现。I-GSD中常见的高脂血症也可能加速肾小球硬化进程。
