Bérard E, Sarles J, Triolo V, Gagnadoux M F, Wernert F, Hadchouel M, Niaudet P
Service de Pédiatrie, Hôpital de l'Archet, CHU de Nice, France.
Pediatr Nephrol. 1998 Feb;12(2):121-4. doi: 10.1007/s004670050418.
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.
阿拉吉耶综合征（AS）的特征是以下五种异常中至少有三种同时出现：慢性胆汁淤积、外周肺动脉狭窄、椎弓缺损、角膜后胚胎环和典型面容。除泌尿系统异常外，AS患者还出现了肾小管间质性肾炎、肾小管酸中毒和系膜脂质沉积。这些肾脏病变的常见表现很少包括高血压。我们报告了5例患者，他们具有AS的五项主要特征中的至少四项，在初次诊断AS后3.5至28年出现了肾血管性继发高血压。血管造影显示所有5例患者均存在单侧或双侧肾动脉狭窄以及主要动脉的各种其他异常：主动脉（3例）、腹腔动脉（4例）、肠系膜上动脉（1例）、锁骨下动脉（1例）。我们的研究结果强调了对AS患者进行动脉血压监测的重要性。如果出现高血压，应寻找肾血管性病因。这些患者中似乎是AS特征的弥漫性血管异常应促使对AS患者的血管异常进行更大规模的研究。
