Document Detail

Clinical features and management of arterial hypertension in children with Williams-Beuren syndrome.
MedLine Citation:
PMID:  19815602     Owner:  NLM     Status:  MEDLINE    
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.
Karim Bouchireb; Olivia Boyer; Damien Bonnet; Francis Brunelle; Stephane Decramer; Gilbert Landthaler; Aurelia Liutkus; Patrick Niaudet; R?mi Salomon
Publication Detail:
Type:  Journal Article; Multicenter Study     Date:  2009-10-08
Journal Detail:
Title:  Nephrology, dialysis, transplantation : official publication of the European Dialysis and Transplant Association - European Renal Association     Volume:  25     ISSN:  1460-2385     ISO Abbreviation:  Nephrol. Dial. Transplant.     Publication Date:  2010 Feb 
Date Detail:
Created Date:  2010-01-22     Completed Date:  2010-05-19     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  8706402     Medline TA:  Nephrol Dial Transplant     Country:  England    
Other Details:
Languages:  eng     Pagination:  434-8     Citation Subset:  IM    
Department of Pediatric Nephrology, Centre de R?f?rence des Maladies R?nales H?r?ditaires de l'Enfant et de l'Adulte, H?pital Necker-Enfants Malades, Paris.
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MeSH Terms
Child, Preschool
Hypertension / diagnosis,  etiology*,  therapy
Retrospective Studies
Williams Syndrome / complications*
Comment In:
Nephrol Dial Transplant. 2010 Feb;25(2):339-41   [PMID:  19934077 ]

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