Moragas A, Huguet P, Torán N, Rona V
Pathol Res Pract. 1983 Mar;176(2-4):176-84. doi: 10.1016/S0344-0338(83)80008-9.
A 9-day-old girl was admitted to hospital because of respiratory distress. The girl died on the 11th day of life shortly after an haemodynamic study and the autopsy disclosed a pulmonary veno-occlusive disease with lesions in relatively early stages of development. Morphological pattern and computerized image analysis data induce to rule out a thrombosis as a previous event to the development of fibrotic changes and point to a "primary" intimal involvement, presumably triggered by an endothelial injury, determining a myxoid change of the intima and leading to a cellular proliferation and ultimate fibrotic occlusion of the small pulmonary veins. Apparently the lesions progress from the smaller pulmonary veins to the larger ones, in which the involvement is focal in character and related to the openings of collateral branches. The homogeneous character of the more recent lesions in the larger veins is in contrast to the heterogeneity of the fibrotic process in the smaller ones.
一名9天大的女婴因呼吸窘迫入院。该女婴在出生后第11天进行血流动力学研究后不久死亡，尸检显示为肺静脉闭塞性疾病，处于相对早期的病变阶段。形态学模式和计算机图像分析数据排除了血栓形成是纤维化改变之前的事件，并指向“原发性”内膜受累，推测是由内皮损伤引发，导致内膜黏液样改变，进而引起细胞增殖，最终导致小肺静脉纤维化闭塞。显然，病变从小肺静脉发展到较大的肺静脉，较大肺静脉的受累为局灶性，与侧支分支的开口有关。较大静脉中较新病变的均匀特征与较小静脉中纤维化过程的异质性形成对比。
