title: Clinical features and management of arterial hypertension in children with Williams-Beuren syndrome.
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.
authors.affiliation: Department of Pediatric Nephrology, Centre de Référence des Maladies Rénales Héréditaires de l'Enfant et de l'Adulte, Hôpital Necker-Enfants Malades, Paris.
authors.forename: Karim
authors.initials: K
authors.lastname: Bouchireb
authors.forename: Olivia
authors.initials: O
authors.lastname: Boyer
authors.forename: Damien
authors.initials: D
authors.lastname: Bonnet
authors.forename: Francis
authors.initials: F
authors.lastname: Brunelle
authors.forename: Stephane
authors.initials: S
authors.lastname: Decramer
authors.forename: Gilbert
authors.initials: G
authors.lastname: Landthaler
authors.forename: Aurelia
authors.initials: A
authors.lastname: Liutkus
authors.forename: Patrick
authors.initials: P
authors.lastname: Niaudet
authors.forename: Rémi
authors.initials: R
authors.lastname: Salomon
mesh_terms: D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D006973:Hypertension / Q000175:diagnosis / Q000209:etiology* / Q000628:therapy; D007223:Infant; D008297:Male; D012189:Retrospective Studies; D018980:Williams Syndrome / Q000150:complications*