Juul S, Ledbetter D, Wight T N, Woodrum D
Department of Pediatrics, University of Washington School of Medicine, Seattle.
Am J Dis Child. 1990 Feb;144(2):229-33. doi: 10.1001/archpedi.1990.02150260109041.
We describe the occurrence of idiopathic infantile arterial calcinosis in three newborn siblings. Unusual features in this disease include ultrasonographic prenatal diagnosis in the second two siblings and ultrastructural studies of the third patient that might shed light on the pathophysiologic characteristics of this disease. Ruthenium-red staining for proteoglycans showed a clearly abnormal structure for the granules in areas of calcification. The granules appeared disorganized, with loss of their normal honeycomb interconnections. They also varied in size and density. In addition, matrix vesicles that might serve as nucleation sites for crystalline calcium phosphate were observed in zones of calcification.
我们描述了三例患有特发性婴儿动脉钙化症的新生儿同胞的病例。该疾病的异常特征包括后两例同胞通过超声进行产前诊断,以及对第三例患者进行的超微结构研究,这些研究可能有助于揭示该疾病的病理生理特征。蛋白聚糖的钌红染色显示钙化区域的颗粒结构明显异常。颗粒呈现无序状态,失去了正常的蜂窝状相互连接。它们的大小和密度也各不相同。此外,在钙化区域观察到可能作为磷酸钙晶体成核位点的基质小泡。