| Clinical features and management of arterial hypertension in children with Williams-Beuren syndrome. | |
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MedLine Citation:
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PMID: 19815602 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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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. |
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Authors:
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Karim Bouchireb; Olivia Boyer; Damien Bonnet; Francis Brunelle; Stephane Decramer; Gilbert Landthaler; Aurelia Liutkus; Patrick Niaudet; R?mi Salomon |
Publication Detail:
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Type: Journal Article; Multicenter Study Date: 2009-10-08 |
Journal Detail:
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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:
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Created Date: 2010-01-22 Completed Date: 2010-05-19 Revised Date: - |
Medline Journal Info:
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Nlm Unique ID: 8706402 Medline TA: Nephrol Dial Transplant Country: England |
Other Details:
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Languages: eng Pagination: 434-8 Citation Subset: IM |
Affiliation:
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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. |
Export Citation:
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| MeSH Terms | |
Descriptor/Qualifier:
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Adolescent Child Child, Preschool Female Humans Hypertension / diagnosis, etiology*, therapy Infant Male Retrospective Studies Williams Syndrome / complications* |
| Comments/Corrections | |
Comment In:
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Nephrol Dial Transplant. 2010 Feb;25(2):339-41
[PMID:
19934077
]
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From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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