Bharati S, Serratto M, DuBrow I, Paul M H, Swiryn S, Miller R A, Rosen K, Lev M
Pediatr Cardiol. 1982;2(1):25-32. doi: 10.1007/BF02265613.
We report our findings in the microscopic examination of the conduction system in four infants with glycogen storage disease, one of whom had adequate electrophysiologic studies. The electrophysiologic studies in the latter case showed P-A and A-H intervals at the lower limits of normal, but the H-V interval was just above the normal mean. This suggests that the rapid conduction was not localized in the anatomic counterpart of the H-V interval. The short P-R interval in the ECG may be related to the enlargement of cells, which may in turn be related to increased glycogen content. The relationship of glycogen per se to the speed of conduction is unknown. We found that the summit of the ventricular septum bulged, probably because of the generally increased cell size, and that the topography of the atrioventricular conducting system was different from normal. This is possibly related both to an increase in the cell sizes of the specialized conducting tissue itself and to deforming effects of this bulging summit of the ventricular septum. New microscopic details of the components of the conducting system are described in these cases.
我们报告了对4例糖原贮积病婴儿传导系统进行显微镜检查的结果，其中1例进行了充分的电生理研究。后1例的电生理研究显示P-A和A-H间期在正常下限，但H-V间期略高于正常均值。这表明快速传导并非局限于H-V间期的解剖对应部位。心电图中短P-R间期可能与细胞增大有关，而细胞增大可能又与糖原含量增加有关。糖原本身与传导速度的关系尚不清楚。我们发现室间隔顶部隆起，可能是因为细胞普遍增大，且房室传导系统的形态与正常不同。这可能既与特殊传导组织本身细胞大小增加有关，也与室间隔顶部隆起的变形作用有关。这些病例中描述了传导系统各组成部分新的微观细节。
