Renovascular hypertension and vascular anomalies in Alagille syndrome
Renovascular hypertension and vascular anomalies in Alagille syndrome
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DOI：
10.1007/s004670050418
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发表时间：
1998-02-01
影响因子：
3
通讯作者：
Niaudet, P
中科院分区：
文献类型：
--
作者：
Bérard, E;Sarles, J;Niaudet, P
Alagille综合征（AS）的特征是至少有以下五种异常中的三种相关：慢性胆汁淤积、外周肺动脉狭窄、椎弓缺损、胚胎毒素和典型面容。除了泌尿系统的异常，肾小管间质性肾炎，肾小管酸中毒，系膜脂质沉积症已注意到在AS，这种肾脏病理的常见表现很少包括高血压。我们报告了5例AS患者，他们至少具有AS的5个主要特征中的4个，在最初诊断为AS后3.5-28年发生继发性肾血管性高血压。5例患者血管造影均显示单侧或双侧肾动脉狭窄及其他主要动脉异常：主动脉3例，腹腔动脉4例，上级肠系膜动脉1例，锁骨下动脉1例。我们的研究结果强调了动脉血压监测在AS患者中的价值。如果发生高血压，应寻找肾血管起源。弥漫性血管异常似乎是这些患者AS的一个特征，应促进对AS血管异常的更大研究。
Alagille syndrome (AS) is characterized by the association of at least three of the following five abnormalities: chronic cholestasis, peripheral pulmonary artery stenosis, vertebral arch defects, embryotoxon, and typical facies. In addition to urological abnormalities, tubulointerstitial nephritis, renal tubular acidosis, and mesangiolipidosis have been noted in AS, The usual manifestations of such renal pathologies rarely include hypertension. We report five patients with at least four of the five major features of AS who developed secondary hypertension of renovascular origin 3.5-28 years after the initial diagnosis of AS. Angiography demonstrated uni- or bilateral renal artery stenosis and various other abnormalities of the main arteries in all five patients: aorta (3 cases), celiac artery (4 cases), superior mesenteric artery (1 case), subclavian artery (1 case). Our findings underscore the value of arterial blood pressure monitoring in patients with AS. If hypertension occurs, a renovascular origin should be sought, The diffuse vascular abnormalities which appeared to be a feature of AS in these patients should prompt larger studies of vascular abnormalities in AS.