THE HEART IN TANGIER DISEASE

一例72岁丹吉尔患者的心脏尸检发现 血浆总胆固醇水平平均为70毫克/分升，低- 密度脂蛋白胆固醇45 mg/dl和高密度脂蛋白胆固醇 胆固醇1.4毫克/分升，谁接受了冠状动脉搭桥术 严重的动脉粥样硬化性冠状动脉疾病。在尸检时，24%(33%) 四大(右、左、左、前)的72个5 mm节段 降支和左回旋支)冠状动脉和4支(15%) 27个5 mm的大隐静脉主-冠脉搭桥管 动脉粥样硬化使横截面积缩小75%以上 斑块。斑块以纤维组织为主(91-97%)。 油红O染色、偏振光显微镜和电子显微镜 显示斑块和壁上有胆固醇沉积 冠状动脉、大隐静脉移植物和大动脉。这类存款也 在组织细胞起源的泡沫细胞、成纤维细胞中均有发现 心脏瓣膜和心脏神经的雪旺细胞。

Cardiac necropsy findings are described in a 72-year-old man with Tangier disease whose plasma total cholesterol levels averaged 70 mg/dl, low- density lipoprotein cholesterol 45 mg/dl, and high-density lipoprotein cholesterol 1.4 mg/dl, and who had coronary artery bypass grafting for severe atherosclerotic coronary artery disease. At necropsy, 24 (33%) of the 72 five-mm segments of the 4 major (right, left main, left anterior descending and left circumflex) native coronary arteries and 4 (15%) of the 27 five-mm segments of the saphenous vein aorto-coronary bypass conduits were narrowed greater than 75% in cross-sectional area by atherosclerotic plaques. The plaques were composed primarily (91-97%) of fibrous tissue. Oil red O staining, polarized light microscopy, and electron microscopy showed the presence of cholesterol deposits in the plaques and in the walls of coronary arteries, saphenous vein grafts and aorta. Such deposits also were found in foam cells of histiocytic origin, fibroblasts in all 4 cardiac valves and in Schwann cells of cardiac nerves.