Clinical features and management of arterial hypertension in children with Williams-Beuren syndrome.
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.
Bouchireb
Karim
K
Department of Pediatric Nephrology, Centre de Référence des Maladies Rénales Héréditaires de l'Enfant et de l'Adulte, Hôpital Necker-Enfants Malades, Paris.
Boyer
Olivia
O
Bonnet
Damien
D
Brunelle
Francis
F
Decramer
Stephane
S
Landthaler
Gilbert
G
Liutkus
Aurelia
A
Niaudet
Patrick
P
Salomon
Rémi
R
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*