Retinal vaso-occlusion in sickling hemoglobinopathies.
Retinal vaso-occlusion in sickling hemoglobinopathies.
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镰状血红蛋白病中的视网膜血管闭塞。
DOI：
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发表时间：
1976
期刊：
影响因子：
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通讯作者：
M. Goldberg
中科院分区：
文献类型：
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作者：
M. Goldberg
镰状细胞性视网膜病的所有表现均代表小动脉和毛细血管闭塞的影响。循环全血粘度增加加上单个镰状红细胞的微栓塞作用导致血管闭塞。氧合作用减少和酸中毒加剧，导致进一步镰状化和进一步血管闭塞。全身镰刀形特征的红斑淤积周期也适用于视网膜。眼睛的透明介质允许直接观察血管闭塞，血管闭塞优先发生在黄斑内和黄斑周围以及视网膜的远周。许多闭塞发作都是短暂的。这些动态事件同时发生在身体的其他部位，但只能在眼睛中看到。视网膜的净效应是其脉管系统的重塑，一些血管关闭，另一些血管重新打开。视网膜中的小动脉开始闭合后，受影响的血管开始自发的、自然演变的过程，导致小动脉吻合、新生血管增殖、玻璃体出血和视网膜脱离。增殖性镰状视网膜病变的晚期阶段最常见于 SC 疾病和 Sthal，可能是因为这两种形式的镰状视网膜病变的全血粘度明显高于正常的全血粘度。视网膜血管闭塞也可能导致爆裂性出血，可能演变成鲑鱼斑、虹彩斑、裂隙性空洞和黑色旭日纹。在某些方面，镰状视网膜病变是独特的，但其许多表现与糖尿病、AC血红蛋白病、Takayasu无脉病、结节病、慢性粒细胞性白血病、视网膜分支静脉阻塞、晶状体后纤维增生和Eales病中发现的视网膜病变相似。
Sickle cell retinopathy, in all of its manifestations, represents the effects of arteriolar and capillary occlusions. Increased viscosity of circulating whole blood plus the microembolic action of individual sickled erythrocytes contribute to vasoocclusion. Decreased oxygenation and increased acidosis develop and lead to further sickling -- and further vaso-occlusion. The cycle of erythrostasis that characterizes sickling throughout the body is also applicable to the retina. The transparent media of the eye permit direct visualization of vaso-occlusions which occur preferentially in and about the macula and in the far periphery of the retina. Many of the occlusive episodes are transient. There dynamic events are simultaneously occurring elsewhere in the body but can only be visualized in the eye. The net effect in the retina is a remodeling of its vasculature, as some vessels close and others reopen. After the onset of arteriolar closure in the retina, affected blood vessels embark on a spontaneous, naturally evolving course of events leading to arteriolarvenular anastomoses, neovascular proliferations, vitreous hemorrhages, and retinal detachment. The advanced stages of proliferative sickle retinopathy are most commonly observed in SC disease and in Sthal, possibly because these two forms of sickling have significantly higher than normal whole blood viscosity. Retinal vaso-occlusions can also lead to blow-out hemorrhages which may evolve into salmon patches, iridescent spots, schisis cavities, and black sunbursts. In some respects sickle retinopathy is unique, but many of its manifestations are similar to those of retinopathies found in diabetes mellitus, AC hemoglobinopathy, Takayasu pulseless disease, sarcoidosis, chronic myelogenous leukemia, branch retinal vein occlusion, retrolental fibroplasia, and Eales disease.