Hypertension Unit and National Referral Centre for Rare Vascular Diseases, Université Paris-Descartes, Assistance Publique Hôpitaux de Paris, Hôpital Européen Georges Pompidou, Paris, France.
J Hypertens. 2012 Jul;30(7):1300-6. doi: 10.1097/HJH.0b013e3283531e1f.
Alagille syndrome is a rare congenital multisystem disorder that may involve heart disease and pulmonary or peripheral artery stenosis. We report the clinical and radiological presentation of five adult patients with Alagille syndrome, hypertension and renal artery stenosis. All had systolic hypertension and a narrowing of the abdominal aorta, corresponding to a secondary midaortic syndrome. Renovascular disease progressed during follow-up, with increases in blood pressure, decreases in glomerular filtration rate and/or kidney atrophy. A literature review identified several anecdotal reports of Alagille syndrome associated with hypertension, renal artery stenosis and/or midaortic syndrome. We discuss this condition, focusing on diagnosis, differential diagnosis, associated conditions and management. Cardiologists, nephrologists and radiologists should be aware of this rare cause of renovascular hypertension and of the need for clinical, biological and echographic follow-up.
肝内胆管发育不良综合征是一种罕见的先天性多系统疾病，可能涉及心脏病和肺动脉或外周动脉狭窄。我们报告了 5 例肝内胆管发育不良综合征、高血压和肾动脉狭窄的成年患者的临床和影像学表现。所有患者均有收缩期高血压和腹主动脉狭窄，符合继发性中主动脉综合征。肾血管疾病在随访过程中进展，血压升高，肾小球滤过率下降和/或肾脏萎缩。文献回顾发现了一些关于肝内胆管发育不良综合征与高血压、肾动脉狭窄和/或中主动脉综合征相关的偶发报告。我们讨论了这种情况，重点是诊断、鉴别诊断、相关疾病和治疗。心脏病专家、肾病专家和放射科医生应该意识到这种罕见的肾血管性高血压的原因，以及进行临床、生物学和超声随访的必要性。
