Lüscher T F, Essandoh L K, Lie J T, Hollier L H, Sheps S G
Mayo Clin Proc. 1987 Mar;62(3):223-9. doi: 10.1016/s0025-6196(12)62447-1.
The association of the Ehlers-Danlos syndrome with many cardiovascular abnormalities is well known. To our knowledge, however, renovascular hypertension due to renal arterial aneurysms has not previously been reported in patients with this disease. We describe a patient with type IV Ehlers-Danlos syndrome who had multiple systemic and bilateral renal arterial aneurysms and hypertension. The hypertension could have been related to compression of renal tissue or arteries (or both) by the large aneurysms, associated stenoses, or small peripheral renal infarctions. A review of all patients in whom the Ehlers-Danlos syndrome had been diagnosed at our institution between 1967 and 1985 revealed that 4 of the 200 patients had hypertension. Three of these patients, and probably all four, had secondary causes of hypertension: Cushing's syndrome, renal insufficiency, or renovascular hypertension (in the present case). We conclude that hypertension is rare in patients with the Ehlers-Danlos syndrome and that, if present, it most likely is secondary hypertension. A thorough search for secondary causes of hypertension should be undertaken in these patients.
埃勒斯-当洛综合征与多种心血管异常相关，这是众所周知的。然而，据我们所知，此前尚未有关于患有该疾病的患者因肾动脉动脉瘤导致肾血管性高血压的报道。我们描述了一名患有IV型埃勒斯-当洛综合征的患者，该患者有多个全身性及双侧肾动脉动脉瘤和高血压。高血压可能与大动脉瘤、相关狭窄或小的外周肾梗死对肾组织或动脉（或两者）的压迫有关。对1967年至1985年间在我们机构被诊断为埃勒斯-当洛综合征的所有患者进行回顾发现，200名患者中有4名患有高血压。其中3名患者，可能所有4名患者，都有高血压的继发原因：库欣综合征、肾功能不全或肾血管性高血压（本病例）。我们得出结论，埃勒斯-当洛综合征患者中高血压较为罕见，若存在，很可能是继发性高血压。应对这些患者进行全面检查以寻找高血压的继发原因。
