Clinical features and management of arterial hypertension in children with Williams-Beuren syndrome
Clinical features and management of arterial hypertension in children with Williams-Beuren syndrome
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DOI：
10.1093/ndt/gfp522
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发表时间：
2010-02-01
影响因子：
6.1
通讯作者：
Salomon, Remi
中科院分区：
文献类型：
--
作者：
Bouchireb, Karim;Boyer, Olivia;Salomon, Remi
方法.本回顾性研究的目的是回顾全身性高血压的临床表现在WBS儿童，其起源和管理。我们纳入了41名确诊为WBS的儿童，他们因高血压被转诊到儿科肾脏科或心脏科。诊断高血压的平均年龄为4.7岁。在41例患者中，24例患者的收缩压（BP）在第95百分位数（1.645 SD）以上+10和+30 mmHg之间，20/41例患者的舒张压在第95百分位数（1.645 SD）和> 10 mmHg之间。39名患者无症状。在17/41例患者中进行的动脉造影显示10例患者（58%）存在肾动脉狭窄（RAS）。所有患者均行超声心动图检查，4例患者（9%）显示峡部缩窄。一半患者（22/41）使用钙通道阻滞剂，在大多数情况下似乎控制了高血压。5例RAS患者接受了介入治疗（3例血管成形术和2例外科搭桥术）。它控制了1例患者的高血压，但对其他4例患者无效。在大多数情况下，药物治疗主要是钙阻滞剂改善了高血压。RAS的介入治疗并不令人鼓舞。
Methods. The aim of this retrospective study was to review the clinical presentation of systemic hypertension in WBS children, its origin and management. We included 41 children with confirmed WBS who were referred to the paediatric nephrology or cardiology unit for hypertension.Results. The mean age at diagnosis of hypertension was 4.7 years. Out of 41, 24 patients had systolic blood pressure (BP) between +10 and +30 mmHg above the 95th percentile (1.645 SD), and 20/41 patients had diastolic BP between the 95th percentile (1.645 SD) and > 10 mmHg. Thirty-nine patients were asymptomatic. Arteriography, performed in 17/41 patients, revealed a renal artery stenosis (RAS) in 10 patients (58%). Echocardiography was performed in all patients and showed isthmic coarctation in four patients (9%). Calcium channel blockers were used in half of the patients (22/41) and seemed to control hypertension in most cases. Interventional treatment of RAS was performed in five patients (three angioplasty and two surgical bypass). It controlled hypertension in one patient but remained ineffective in the four others.Conclusions. Medical treatment essentially calcium blockers improved hypertension in most cases. Interventional treatment of RAS has not been encouraging.