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NDT Advance Access published online on October 8, 2009
Nephrology Dialysis Transplantation, doi:10.1093/ndt/gfp522
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© The Author 2009. Published by Oxford University Press [on behalf of ERA-EDTA]. All rights reserved. For Permissions, please e-mail: journals.permissions@oxfordjournals.org


Clinical features and management of arterial hypertension in children with Williams–Beuren syndrome

Karim Bouchireb1, Olivia Boyer1,2, Damien Bonnet3, Francis Brunelle4, Stephane Decramer5, Gilbert Landthaler6, Aurelia Liutkus7, Patrick Niaudet1,2 and Rémi Salomon1,2

1 Department of Pediatric Nephrology, Centre de Référence des Maladies Rénales Héréditaires de l’Enfant et de l’Adulte, Hôpital Necker–Enfants Malades, Paris 2 Université Paris Descartes, Faculté de médecine, France 3 Department of Pediatric Cardiology 4 Department of Pediatric Radiology, Hôpital Necker–Enfants Malades, Paris 5 Department of Pediatric Nephrology, Hôpital D’Enfants, Toulouse 6 Department of Pediatric Nephrology, Hôpital Charles Nicolle, Rouen 7 Department of Pediatric Nephrology, Hôpital Femmes Mère Enfants, Lyon, France

Correspondence and offprint requests to: Rémi Salomon; E-mail: remi.salomon{at}nck.aphp.fr



  Abstract

Background. Hypertension is a common finding in children with Williams–Beuren syndrome (WBS).

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.

Keywords: arterial hypertension; renal artery stenosis; Williams–Beuren syndrome

Received for publication: 5. 7.09
Accepted in revised form: 7. 9.09


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