Perloff J K, Latta H, Barsotti P
Department of Medicine, and the Ahmanson Adult Congenital Heart Disease Center, University of California at Los Angeles, USA.
Am J Cardiol. 2000 Dec 1;86(11):1198-204. doi: 10.1016/s0002-9149(00)01202-9.
We present evidence of 2 distinct glomerular abnormalities in cyanotic congenital heart disease--vascular and nonvascular--each believed to reflect a distinct pathogenesis. Glomeruli from both kidneys were studied with light microscopy in 13 necropsied cyanotic patients and in 8 controls. The vascular study characterized hilar arteriolar dilatation, capillary diameter, glomerular diameter, and capillary engorgement with red blood cells. The nonvascular study characterized juxtaglomerular cellularity, mesangeal cellularity, mesangeal matrix, focal interstitial fibrosis, and megakaryocytic nuclei per cm2 of renal cortex. There was a significant increase in each of the above vascular and nonvascular items of interest relative to controls. Electron microscopy identified whole megakaryocytes with their cytoplasm in glomeruli. The vascular abnormality is believed to result from intraglomerular release of nitric oxide. The nonvascular abnormality is believed to result from platelet-derived growth factor and transforming growth factor-beta.
我们提供了关于紫绀型先天性心脏病中两种不同肾小球异常的证据——血管性和非血管性——每种异常都被认为反映了不同的发病机制。对13例尸检的紫绀型患者和8例对照者的双侧肾脏肾小球进行了光学显微镜研究。血管性研究对肾门小动脉扩张、毛细血管直径、肾小球直径以及红细胞引起的毛细血管充血进行了特征描述。非血管性研究对肾小球旁细胞数量、系膜细胞数量、系膜基质、局灶性间质纤维化以及每平方厘米肾皮质中的巨核细胞核进行了特征描述。相对于对照组，上述血管性和非血管性相关指标均显著增加。电子显微镜在肾小球中发现了带有细胞质的完整巨核细胞。血管异常被认为是由肾小球内一氧化氮释放所致。非血管异常被认为是由血小板衍生生长因子和转化生长因子-β所致。
