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Query: UMLS:C0020538 (hypertension)
 170,190 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Four patients with suprarenal coarctation of the abdominal aorta were managed from 1978 to 1993 (mean follow-up 8.75 years). Ages at the time of diagnosis were 2 months, 8 months, 4.5 years, and 15 years, respectively. Three children presented with severe hypertension, two of whom were in congestive heart failure, and the fourth child presented with a cold, ischemic leg. The 8-month-old patient had Williams syndrome (supravalvular aortic and pulmonic stenosis, bilateral renal artery stenosis and celiac artery occlusion, "elfin" facies, and mental retardation) and was treated nonoperatively. After 12 years of follow-up, he was given five medications to control hypertension, cardiac arrhythmias, and heart failure. Three patients with abdominal aortic coarctation were treated operatively and none died. Two patients underwent bypass grafting from the supraceliac aorta to the infrarenal aorta, with bilateral renal artery reconstruction in one. Postoperative arteriograms obtained 1 year or more after operation were normal in both cases. The 2-month-old patient underwent patch aortoplasty, with subsequent reoperation 1.5 years later for recurrent hypertension and heart failure with a bypass graft to the left kidney and removal of an infarcted right kidney. In all three patients, operative repair of the suprarenal aortic coarctation has resulted in long-term control of blood pressure and cardiac and renal function.
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PMID:Coarctation of the abdominal aorta. 852 35

Mid-aortic syndrome (MAS) is an uncommon condition characterized by segmental narrowing of the proximal abdominal aorta and ostial stenosis of its major branches. It is usually diagnosed in young adults, but may present in childhood as a challenging problem. Over the past 20 years 13 patients with MAS have presented to this institution. All had hypertension, four had associated neurofibromatosis, three persistent eosinophilia and three had Williams syndrome. In all cases arteriography showed a smooth segmental narrowing of the abdominal aorta with concomitant stenosis at the origins of the renal arteries. Six children were successfully treated with antihypertensive medication alone. Percutaneous transluminal angioplasty was attempted in two cases with poor result. Surgery was indicated in seven children with refractory hypertension and progressive renal impairment. Techniques used to revascularize the kidneys included thoracoabdominal to infrarenal aortic bypass with renal artery reimplantation, splenorenal bypass, gastroduodenal to renal bypass, aortorenal bypass and autotransplantation.
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PMID:Mid-aortic syndrome presenting in childhood. 868 75

Kidneys and urinary tract were examined systematically by ultrasonography in 130 patients with Williams-Beuren syndrome (59 females, median age 5.5 years; 71 males, median age 6.4 years). In addition, serum creatinine was determined and an analysis was performed. Creatinine clearance was available in 79 patients. Renal angiographic examinations were done in 18 patients, 8 of whom had renal artery narrowing (44%). The incidence of renal anomalies in Williams-Beuren syndrome was 17.7% vs. around 1.5% in the normal population (P < 0.0003). The spectrum of these anomalies ranged from minor anomalies such as bladder diverticula to more severe malformations such as renal aplasia or hypoplasia (in 5 of 130 patients). In nine patients a duplicated kidney was found. A decreased creatinine clearance (two patients), recurrent symptomatic urinary tract infections (four patients), and hypertension were uncommon. Nephrocalcinosis was not found in our patients. Our data demonstrate that the risk of a structural abnormality of the kidneys and the urinary tract is increased 12- to 36-fold in Williams-Beuren syndrome compared to the normal population. Ultrasound screening of the renal system should be part of the first evaluation of WBS patients.
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PMID:Incidence and spectrum of renal abnormalities in Williams-Beuren syndrome. 872 24

The prevalence of hypertension and the diurnal blood pressure pattern were investigated in patients with Williams-Beuren syndrome (WBS) by blood pressure measurements in 142 children, adolescents, and young adults (female n = 62, male n = 80; median age 6.5 years (0.1-34.3 years)) and evaluation of ambulatory blood pressure data from 45 patients (female n = 21, male n = 24; median age 7.8 years (1-23.8 years)). Measurements revealed systolic hypertension in 46.5% of 142 patients, diastolic hypertension occurred in 36.6% (i.e. actual pressure > 95 percentile). According to the ambulatory data 42.2% of 45 patients had hypertension (mean arterial pressure > normal + 2SD). The nocturnal decline of the blood pressure was normal in hypertensive patients but reduced in normotensives (p < 0.01 vs normals). Males were more often hypertensive than females (46% vs 38%). Hypertensives had a higher body mass index than normotensives (19.5 vs 16.6 kg/m2, p < 0.05). In normo- and hypertensive WBS patients mean heart rates were elevated during day- and nighttime (p < 0.02 vs normals) the latter due to a reduced nocturnal decline. The prevalence of hypertension in WBS patients amounts to about 40%, thus being four- to eight-fold in comparison to healthy young adults or children. The diurnal blood pressure pattern and the elevated heart rates indicate that an increased arterial stiffness due to the vascular disease in the WBS and augmented sympathetic activity might play a role in the genesis of hypertension. Thus, effective antihypertensive treatment is likely to become difficult. From our experience beta-blocking agents are often successful in hypertensive WBS patients.
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PMID:[Arterial hypertension and blood pressure profile in patients with Williams-Beuren syndrome]. 923 96

Twenty percutaneous transluminal renal angioplasties were performed on 16 children (mean age 8.7 years) with hypertension secondary to renal artery stenosis (RAS). The aetiologies were neurofibromatosis (n = 1), Williams syndrome (n = 2), Takayasu arteritis (n = 1) and fibromuscular dysplasia (n = 12). The stenosis was isolated proximal or distal in 13 cases and multiple in 3 cases. Angioplasty resulted in a complete cure without medical treatment in 9 cases. Angioplasty allowed a reduction of medical treatment in two single stenoses, but was ineffective in all cases of multiple stenoses. Our findings show that angioplasty of RAS in children is an effective treatment when the stenosis is isolated, with a 69 % success rate. It seems ineffective in case of multiple stenoses (three cases).
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PMID:Percutaneous transluminal angioplasty of renal artery stenosis in children. 942 77

Previous studies report conflicting frequencies of hypertension in cohorts of patients with Williams syndrome (WS). We studied blood pressure (BP) in WS using 24-hour ambulatory BP monitoring. This technique reliably measures day- and nighttime BP in a subject's natural environment and provides better prognostic information on long-term risks of hypertension than casual BP determinations. Twenty WS subjects evaluated through a multidisciplinary WS clinic and 35 age and gender-matched controls were studied. We found that WS subjects had significantly higher ambulatory BP than controls. After controlling for age, sex, and weight, the diagnosis of WS added approximately 10 mmHg to mean daytime and nighttime BPs. Hypertension, as defined by elevated mean daytime BP, was present in 40% of WS subjects versus 14% of controls (P < 0.05); among the children studied this difference was even more dramatic with 46% of WS children versus 6% of control children classified as hypertensive (P = 0.01). We also demonstrated normal diurnal BP variation but no evidence of a "white coat" effect or increased BP variability. Interestingly, parental reporting of a history of infantile hypercalcemia was strongly associated with the presence of hypertension (P = 0.008). Our data demonstrate that both children and adults with WS have higher mean BP and higher frequency of hypertension than healthy controls. Thus, elevated BP readings in the office setting should not be dismissed but require more thorough assessment.
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PMID:Elevated ambulatory blood pressure in 20 subjects with Williams syndrome. 1023 42

The purpose of a neuroanatomical analysis of Williams Syndrome (WMS) brains is to help bridge the knowledge of the genetics of this disorder with the knowledge on behavior. Here, we outline findings of cortical neuroanatomy at multiple levels. We describe the gross anatomy with respect to brain shape, cortical folding, and asymmetry. This, as with most neuroanatomical information available in the literature on anatomical-functional correlations, links up best to the behavioral profile. Then, we describe the cytoarchitectonic appearance of the cortex. Further, we report on some histometric results. Finally, we present findings of immunocytochemistry that attempt to link up to the genomic deletion. The gross anatomical findings consist mainly of a small brain that shows curtailment in the posterior-parietal and occipital regions. There is also subtle dysmorphism of cortical folding. A consistent finding is a short central sulcus that does not become opercularized in the interhemispheric fissure, bringing attention to a possible developmental anomaly affecting the dorsal half of the hemispheres. There is also lack of asymmetry in the planum temporale. The cortical cytoarchitecture is relatively normal, with all sampled areas showing features typical of the region from which they are taken. Measurements in area 17 show increased cell size and decreased cell-packing density, which address the issue of possible abnormal connectivity. Immunostaining shows absence of elastin but normal staining for Lim-1 kinase, both of which are products of genes that are part of the deletion. Finally, one serially sectioned brain shows a fair amount of acquired pathology of microvascular origin related most likely to underlying hypertension and heart disease.
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PMID:V. Multi-level analysis of cortical neuroanatomy in Williams syndrome. 1095 35

The neurotrophin nerve growth factor (NGF) is a major regulator of peripheral and central nervous system development. Serum NGF was measured in normally developing control children (n=26) and in individuals affected by congenital syndromes associated with learning disability: either Williams syndrome (WS; n=12) or Down syndrome (DS; n=21). Participants were assessed at three distinct developmental stages: early childhood (2 to 6 years), childhood (8 to 12 years), and adolescence (14 to 20 years). A sample was taken only once from each individual. Serum NGF levels were markedly higher in participants with WS, than DS and control participants. In addition, different developmental profiles emerged in the three groups: while in normally developing individuals NGF levels were higher in early childhood than later on, children with WS showed constantly elevated NGF levels. When compared to control participants, those with DS showed lower NGF levels only during early childhood. Neuropsychological assessment confirmed previously reported differences among the three groups in the development of linguistic/cognitive abilities. Some features of individuals with WS, such as hyperacusis and hypertension, could be related to high-circulating NGF levels.
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PMID:Serum NGF levels in children and adolescents with either Williams syndrome or Down syndrome. 1110 46

The middle aortic syndrome, with diffuse narrowing of the thoracic and abdominal aorta, was present in 10 of 18 patients with Williams' syndrome (55%). There were 3 thoracic coarctations, and 2 abdominal coarctations, with gradients greater than 20 mmHg across the zone of narrowing. Seven patients had mild renal arterial stenosis, and 6 had visceral arterial stenoses. Ten were hypertensive. Measured dimensions of the aortic lumen failed to increase with age in 3 males who had serial angiographic studies. One developed mesenteric arterial stenosis, with mild bilateral renal arterial stenoses, between the ages of 9 and 19 years. Aortic intravascular ultrasound performed in 2 patients confirmed abnormally thick vessel walls with small lumens. Diffusely narrowed and thick-walled stiff arteries, lacking elastin, are a feature of Williams' syndrome. The arteriopathy tends to progress with age, and systemic hypertension is common in teenagers and beyond. The middle aortic syndrome was present in more than half our patients, and does not necessarily reflect a bias because of cardiologic referral. Aortography with measurement of aortic diameters and delineation of the visceral branches is an important requirement for complete evaluation of patients with Williams' syndrome.
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PMID:The middle aortic syndrome: an important feature of Williams' syndrome. 1111 92

The aim of the study was to assess workload capacity and blood pressure (BP) response to treadmill exercise and 24-hour BP monitoring in children with Williams syndrome. Seventeen children were examined (8 males and 9 females) whose mean age was 13.8 +/- 3.6 years. Six patients were on antihypertensive therapy. Each patient underwent clinical examination and measurement of BP at rest, during exercise, and during 24-hour monitoring. Two-dimensional echocardiogram and echo-Doppler of renal arteries were performed. The test was stopped for muscular fatigue or reduced cooperation. The patients, when compared to a population of healthy children, had reduced total time of exercise (7.3 +/- 1.9 vs 14.3 +/- 2.6 min, p < 0.001) and, at the same workload, increased heart rate (167 +/- 19 vs 145 +/- 16 beats/min, p < 0.001) and increased maximum systolic BP (146 +/- 27 vs 128 +/- 12 mmHg, p = 0.01). Ambulatory blood pressure measurement values showed higher systolic blood pressure both during daytime and nighttime. Our study confirms that children and adolescents with Williams syndrome are at high risk for hypertension, probably related to the alterations of large arteries. The data relating to the synthesis of elastin may have a direct relationship to the compliance of the arterial system, leading to hypertension.
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PMID:Exercise testing and 24-hour ambulatory blood pressure monitoring in children with Williams syndrome. 1189 56


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