RESUMO OBJETIVO Descrever a incidência, o diagnóstico e o tratamento da hipertensão arterial sistêmica relacionada com estenose da artéria renal em pacientes com síndrome de Williams-Beuren. MÉTODOS Sessenta e cinco pacientes com síndrome de Williams-Beuren foram avaliados quanto à presença de hipertensão. Os pacientes foram submetidos à ultrassonografia com Doppler das artérias renais e ecocardiograma Doppler. Aqueles com suspeita de hipertensão renovascular foram submetidos à tomografia computadorizada ou angiografia por ressonância magnética da aorta e vasos renais ou angiografia convencional. RESULTADOS A hipertensão arterial sistêmica foi diagnosticada em 21/65 pacientes com síndrome de Williams-Beuren (32%, 13 do sexo masculino), com idade média de 13,9 anos (5 meses-20 anos). Em 8/21 pacientes foi detectada a hipertensão renovascular. Angioplastia não teve sucesso em cinco pacientes com estenose da artéria renal, necessitando de tratamento adicional. O ecocardiograma Doppler mostrou anormalidades cardíacas em 16/21 (76%) pacientes hipertensos. CONCLUSÃO As anormalidades cardíacas e hipertensão arterial em pacientes com síndrome de Williams-Beuren são muito frequentes, sendo necessários uma avaliação minuciosa e seguimento para diminuir o risco cardiovascular e a morbimortalidade desses pacientes

SUMMARY AIM To describe the incidence, diagnosis, and management of systemic arterial hypertension related to renal artery stenosis in patients with Williams-Beuren syndrome. METHODS Sixty-five patients with Williams-Beuren syndrome were evaluated for hypertension. Enrolled patients underwent Doppler sonography of the renal arteries and Doppler echocardiography. Those with Doppler sonography-detected lesions or with normal Doppler sonography but severe hypertension underwent computed tomography or gadolinium-enhanced magnetic resonance angiography of the aorta and renal vessels. Patients needing vascular therapeutic intervention underwent conventional angiography. RESULTS Systemic arterial hypertension was diagnosed in 21/65 patients with Williams-Beuren syndrome (32%; 13 male) with a mean age of 13.9 years (5mo-20yrs). In 8/21 patients renovascular hypertension was detected. Angioplasty was unsuccessful in five patients with renal artery stenosis, requiring additional treatment. Doppler echocardiography showed cardiac abnormalities in 16/21 (76%) hypertensive patients. CONCLUSION Cardiac abnormalities and hypertension in patients with Williams-Beuren syndrome are common. Thus, thorough evaluation and follow-up are necessary to reduce cardiovascular risks and mortality of these patients

OBJETIVO: Avaliar o monitor OMRON 705-CP de medida de pressão arterial em adolescentes e adultos jovens. MÉTODOS: De acordo com o protocolo da British Hypertension Society e da Association for the Advancement of Medical Instrumentation, realizamos a validação do aparelho em 60 adolescentes. O monitor foi conectado em Y com a coluna de mercúrio e foram realizadas 4 medidas consecutivas e simultâneas, analisadas as independentes e calculadas as diferenças médias entre as pressões e o desvio padrão dessas diferenças. Os resultados foram analisados de acordo com o sistema de grau do protocolo utilizado. RESULTADOS: Foram avaliadas 240 medidas. A idade média dos pacientes foi 16,3 anos. Quando comparada a medida realizada pela coluna de mercúrio com o aparelho houve uma diferença < 15 mmHg em 97,9% das medidas sistólicas e 98,8% das diastólicas; uma diferença < 10 mmHg em 86,3% das medidas sistólicas e 90,4% das diastólicas, classificada como grau A; uma diferença < 5 mmHg em 59,1% das medidas sistólicas e 67% das medidas diastólicas, considerada pela classificação entre A/B. A diferença média e o desvio padrão dessa diferença na pressão sistólica foi de 2,91±6,42 mmHg e para a diastólica de 1,16±5,79 mmHg. CONCLUSÃO: O monitor OMRON 705-CP mostrou-se válido para medidas de pressão arterial de adolescentes segundo o protocolo empregado.

OBJECTIVE: To assess the Omron 705-CP monitor for measuring blood pressure in adolescents and young adults. METHODS: According to the protocols of the British Hypertension Society and the Association for the Advancement of Medical Instrumentation, we performed validation of the device in 60 adolescents. The Omron monitor was connected in Y to a mercury column. Four consecutive and simultaneous measurements were taken with the mercury column sphygmomanometer and the test device, were independently analyzed, and the mean differences between the blood pressure measurements and the standard deviations of those differences were calculated. The results were analyzed according to the grading system of the protocol used. RESULTS: Two hundred and forty measurements were evaluated. The mean age of the patients was 16.3 years. When the measurement performed with the mercury column sphygmomanometer was compared with that taken with the device, a difference < 15 mmHg was observed in 97.9% of the systolic and 98.8% of the diastolic blood pressure measurements; a difference < 10 mmHg was observed in 86.3% of the systolic and 90.4% of the diastolic blood pressure measurements, which was classified as grade A; a difference < 5 mmHg was observed in 59.1% of the systolic and 67% of the diastolic blood pressure measurements, and was classified as grade A/B. The mean difference and the standard deviation of that difference for the systolic blood pressure was 2.91±6.42 mmHg, and, for the diastolic blood pressure, it was 1.16±5.79 mmHg. CONCLUSION: The Omron 705-CP monitor proved to be useful for measuring blood pressure in adolescents according to the protocol used.