Walter M, Kerber S, Fechtrup C, Seedorf U, Breithardt G, Assmann G
Institut für Klinische Chemie und Laboratoriumsmedizin, Westfälischen Wilhelms-Universität Münster.
Z Kardiol. 1994 May;83(5):381-5.
A 60-year-old homozygous patient with familial high density lipoprotein deficiency (Tangier disease) was examined by coronary angiography and intravascular ultrasound because of typical angina pectoris. We found a normal left ventricular function, moderately diffuse coronary sclerosis without stenosis, and no critical stenosis of peripheral arteries. Intravascular ultrasound revealed normal thickness and the three-layer appearance of the arterial intima, media, and adventitia within the peripheral arteries, and showed a single, discrete arteriosclerotic lesion in one iliac artery segment. The lack of severe atherosclerosis was remarkable insofar as massive foam cell formation in reticuloendothelial tissues and the virtually complete absence of circulating HDL is characteristic of Tangier disease and had been previously demonstrated in this patient.
一名60岁的家族性高密度脂蛋白缺乏症(丹吉尔病)纯合子患者因典型心绞痛接受了冠状动脉造影和血管内超声检查。我们发现其左心室功能正常,有中度弥漫性冠状动脉硬化但无狭窄,外周动脉无严重狭窄。血管内超声显示外周动脉内膜、中膜和外膜厚度正常且呈三层结构,并在一个髂动脉节段发现单个、离散的动脉硬化病变。鉴于网状内皮组织中大量泡沫细胞形成以及循环HDL几乎完全缺乏是丹吉尔病的特征,且此前已在该患者中得到证实,严重动脉粥样硬化的缺乏颇为显著。