Aneurysms of coronary arteries in a patient with adult polycystic kidney disease: arteriosclerosis or involvement by the primary disease?
Aneurysms of coronary arteries in a patient with adult polycystic kidney disease: arteriosclerosis or involvement by the primary disease?
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成人多囊肾病患者冠状动脉瘤：动脉硬化还是原发病受累？
DOI：
10.1007/bf00179997
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发表时间：
1993
期刊：
影响因子：
--
通讯作者：
M. Wehling
中科院分区：
文献类型：
--
作者：
M. Christ;U. Bechtel;S. Schnaack;Karl Theisen;M. Wehling
成人多囊肾病常伴有胃肠道和心血管异常。包括高血压、二尖瓣脱垂、主动脉根部轻度扩张、腹主动脉瘤、主动脉瓣、二尖瓣和三尖瓣返流的易感性，使人联想到马凡氏综合征。虽然成人型多囊肾病确切的分子机制还没有很好地建立，但假设胶原结构的普遍缺陷。然而，最严重的血管问题是典型的颅内动脉瘤，其蛛网膜下腔出血的发生率高，死亡率高。我们报告一例成人型多囊肾合并压力性心绞痛患者偶然发现冠状动脉扩张。患者的典型心绞痛由左心室肥大和冠心病解释。多发性肝囊肿，二尖瓣脱垂，冠状动脉瘤在这个病人与成人多囊肾病似乎反映了一个广泛的结缔组织疾病在这种综合征的表现。
Adult polycystic kidney disease is frequently associated with gastrointestinal and cardiovascular abnormalities. These include hypertension, mitral valve prolapse, mild dilation of the aortic root, abdominal aneurysms, and predisposition to aortic, mitral, and tricuspidal valve regurgitation reminiscent of Marfan's syndrome. Although tho exact molecular mechanisms of adult polycystic kidney disease are not well established, a generalized defect of collagen structure is hypothesized. The most severe vascular problems, however, are typical intracranial aneurysms with a high incidence of subarachnoid hemorrhage and a high mortality rate. We report a case of dilated coronary arteries found incidentally in a patient with adult polycystic kidney disease and stress-induced angina pectoris. The typical angina pectoris of the patient is explained by left ventricular hypertrophy and coronary heart disease. Multiple liver cysts, mitral valve prolapse, and the coronary aneurysms in this patient with adult polycystic kidney disease appear to reflect the manifestation of a generalized connective tissue disorder in this syndrome.