Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0020538 (hypertension)
 170,190 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Reports of adults with Williams syndrome (WS) have been rare. We have evaluated 13 adult WS patients and reviewed 16 case reports of WS in patients older than age 16 years. Adults in our study had progressive multisystem medical problems. Cardiovascular complications were common (12/13) including hypertension (8), supravalvular aortic stenosis (9), aortic hypoplasia (3), pulmonic artery stenosis (4), peripheral stenoses (3), and mitral valve prolapse (2). Joint limitation (12/13) was progressive, often accompanied by kyphoscoliosis and lordosis. Recurrent urinary tract infections in 6 individuals led to radiologic studies showing urethral stenosis in 2, and bladder diverticula and vesicoureteral reflux in 3. Gastrointestinal problems included obesity (5), chronic constipation (7), diverticulosis (3), and cholelithiasis (4). Hypercalcemia was documented in 5 patients, although others had hypercalcemic symptoms (abdominal pain, polyuria, and constipation). One 45-year-old man had parathyroid hyperplasia. Previous reports likewise document significant morbidity. Thus, Williams syndrome in an adult appears to dictate aggressive evaluation and monitoring. Investigation of calcium metabolism should be undertaken in each adult WS patient.
 ...
PMID:Adults with Williams syndrome. 189 83

The cardiac changes resulting from mechanical overload of the left ventricle have been well documented and a variety of compensatory mechanisms described. These include a decrease in maximum velocity (V0) of shortening in the absence of reduction in active tension (P0), and a reversible decrease in myofibrillar adenosine triphosphatase activity resulting from isoenzymic shift from, predominantly, a form of myosin with high ATPase activity (V1) to another with low (V3). The thermodynamic advantage of the transition is the hypertrophied muscle possesses a more energy-efficient form of contraction. These reversible transitions resulted from altered gene expression of isoenzymic forms of myosin heavy chain. It must be borne in mind that the adaptational modifications just described appear to occur only in smaller animals such as the rat, that possesses several myosin isozymes. In large mammals it is mainly the V3 form of myosin that is present, which does not change with altered contractile state. Responses of the large arteries to hypertension have been poorly studied. This is surprising when one recalls that degenerative disease of such vessels, that include the aorta, carotids and ileo-femoral arteries is almost an obligatory concomitant of hypertension. Such studies as have been carried out indicate that hyperplasia is specific for abdominal aortic stenosis while hypertrophy is found in aortic smooth muscle in rats with systemic hypertension. Mechanically, an increase in V0 with no change in P0 have been reported; an increase in myofibrillar ATPase activity was also reported. Though two myosin heavy chain isozymes have been found in aortic smooth muscle densitometry did not reveal any difference in distribution between tissues from control and hypertensive rats.(ABSTRACT TRUNCATED AT 250 WORDS)
 ...
PMID:Cardiovascular adaptations to mechanical overload. 213 92

Many laboratories have provided evidence to support the concept that LV hypertrophy is associated with many significant coronary perfusion abnormalities. The decreased coronary flow reserve in the presence of LV hypertrophy is implicated as the mechanism of angina in patients with aortic stenosis and normal coronary arteries. The abnormal subendocardial autoregulation found in dogs may have important implications for the abrupt treatment of hypertension in patients with LV hypertrophy. The marked increase in mortality and the wavefront of infarction in dogs with hypertension and LV hypertrophy may have striking clinical implications for the salvage of myocardium in patients with chronic hypertension and LV hypertrophy during acute myocardial infarction.
 ...
PMID:Effects of left ventricular hypertrophy on the coronary circulation. 214 Dec 26

The existence of tissue renin angiotensin system (RAS) has been widely suggested in the recent literature by 2 main approaches: first, a dissociation between antihypertensive effects of angiotensin converting enzyme (ACE) inhibitors and the levels of stimulation of the circulating RAS; secondly, by the demonstration of the presence of the 3 key-proteins of the system (angiotensinogen, creatinine, and converting enzyme) within the 3 main target-organs of hypertension (i.e. kidney, heart and vessels). Those organs are capable to synthetize locally angiotensin II. Ramipril, a new ACE inhibitor (Triatec), which possesses a high affinity for tissue CE of those organs, according to previous publications by Unger, has been used as a tool for the investigations of the inhibition of those systems in human hypertension: a decrease of micro proteinuria has been without antihypertensive effects. In binephrectomized patients, ramipril has been shown to possess an antihypertensive effect. Finally, an important improvement of myocardial hypertrophy has been shown in hypertensive patients. Furthermore, this effect has been observed in animals (rats with aortic stenosis) even with low doses without antihypertensive effects. Further studies with new methodological approaches are still necessary.
 ...
PMID:[Tissue renin-angiotensin system. Physiology and physiopathological value of their inhibition by ramipril]. 214 94

Abnormalities in the diastolic function of the left ventricular pump are the common determinant and, above all, the earliest manifestation of all forms of chronic left ventricular failure, whether or not the left ventricular systolic function is abnormal. Congestive signs, in particular, are directly related to abnormalities of ventricular filling. Primary diastolic dysfunction is the cause of left ventricular failure in about 40 p. 100 of the cases, but it may also be observed in almost all cardiopathies. In myocardial ischaemia the pressure-volume relation is displaced upwards owing to a slowed down, inhomogeneous and incomplete relaxation. Left ventricular hypertrophy, whether it is due to excessive pressure (arterial hypertension, aortic stenosis) or reflects a primary hypertrophic cardiomyopathy, is associated with a slowing down of ventricular relaxation and a reduction of left ventricular diastolic distensibility, even though the ventricular pump systolic function remains normal for a long time. Outside alterations in the distensibility of the ventricular muscle, ventricular dilatation alters ventricular filling by forcing the ventricle to function on the vertical part of its diastolic pressure-volume relation. Nowadays, the aged hearts is the most frequent cause of heart failure with normal systolic function. In all cases dysrhythmias and atrioventricular desynchronization act as aggravating factors. Treatment is often difficult since positively inotropic drugs or arterial vasodilators frequently have a modest or even deleterious effect.
 ...
PMID:[Disorders of diastolic function in chronic left ventricular insufficiency]. 214 34

Extensive bodies of literature describe protein synthesis and processing; the endocrinology and metabolic bases whereby a variety of hormonal, mechanical, and nutritional influences affect cell function and adaptive responses; and various regulatory mechanisms mediating concerted intracellular control. Nonetheless, our current understanding of the mechanisms responsible for the regulation and subordination of protein synthesis to the overall metabolic and stimulus-response status of the cell is inadequate. The endoplasmic reticulum is central to these concerns. Potential roles of the endoplasmic reticulum in the regulation of protein synthesis are largely unexplored. We have attempted in this rather speculative review, based largely on our own data, to project a view of the endoplasmic reticulum as moderating the rate of translation through a mechanism sensitive to sequestered Ca2+. Compensatory routes whereby cells accommodate to Ca2+ deprivation so as to resume reasonable rates of protein synthesis are seen also to focus on the endoplasmic reticulum. With additional research, the underlying relationships that exist among reticular Ca2+ storage, protein processing, and mechanisms of translational control should become more broadly evident. The prevailing view of Ca2+ as a regulator of cytosolic processes may require some extension if sequestered Ca2+ participates in biological control mechanisms emanating from the endoplasmic reticulum. In effect, a reciprocal relationship would presumably exist among processes supported by cytosolic free Ca2+ vs those promoted by sequestered stores of the cation. Speculatively, such reciprocity would allow the rapid diversion of energy from one set of processes to the other. Conceivably, chronic Ca2+ loading at sequestered sites may be related to certain cellular adaptive Ca2+ loading involving tissue hypertrophy. Potential examples of stretch-induced responses that could be cited include thickening of arteriolar smooth muscle walls in hypertension (8) and cardiac hypertrophy in aortic stenosis (46).
 ...
PMID:Calcium-dependent regulation of protein synthesis in intact mammalian cells. 218 68

Long-term performance of Starr-Edwards silastic ball (SESB, n = 168) and St Jude Medical bi-leaflet (SJMB, n = 93) valves in patients who were alive 30 days after implantation (1980-86) for aortic stenosis was compared. Mean follow-up was 3.0 years (0.1-7.9 years). The SESB and SJMB groups differed as regards female gender (18% vs 47%, P less than 0.0001), NYHA classes III-IV (59% vs 72%, P less than 0.05), coronary artery disease (CAD, 32% vs 62%, P less than 0.01) in patients with coronary arteriography (n = 82 and n = 55, respectively), and prosthetic annulus diameter (26 +/- 1 vs 23 +/- 2 mm, P less than 0.0001). Five-year survival +/- SE in SESB vs SJMB patients was: total population, 89 +/- 3% vs 80 +/- 6% (NS); coronary arteriography population, no CAD, 90 +/- 4% vs 100% (NS), and with CAD, 71 +/- 11% vs 60 +/- 13% (NS; P = 0.01 for CAD). Five-year event-free survival +/- SE in SESB vs SJMB patients was 95 +/- 2% vs 97 +/- 2% (NS) for thromboembolism, 95 +/- 2% vs 89 +/- 4% (NS) for coumadin-related haemorrhage, 98 +/- 1% vs 99 +/- 1% (NS) for endocarditis, 98 +/- 1% vs 94 +/- 5% (NS) for paravalvular leak, 88 +/- 3% vs 79 +/- 6% (NS) for all valve-related complications, and 98 +/- 1% vs 95 +/- 4% (NS) for prosthesis replacement. Thrombotic occlusion or structural failure were not observed. No patients without CAD experienced thromboembolic events. Cox regression analyses (in both total population and coronary arteriography population) of survival as well as the various complications revealed that the type of prosthesis did not have predictive influence. CAD was an independent risk factor for thromboembolism, haemorrhage, and all valve-related complications. Previous systemic hypertension was independently predictive of haemorrhage. The SESB and SJMB prostheses showed comparable and acceptable long-term performance. Only patient-related variables, notably CAD, influenced late results. The proven durability and relatively low price of the SESB valves together with the excellent haemodynamic performance of even small-sized SJMB valves should be considered in the light of the present results.
 ...
PMID:Long-term performance of Starr-Edwards silastic ball valves and St Jude Medical bi-leaflet valves. A comparative analysis of implantations during 1980-86 for aortic stenosis. 231 12

Mild-aortic dysplastic syndrome is usually presented with advanced hypertension in young individuals in association with either weak or absent femoral pulses, due to diffuse narrowing of the aorta in its mid thoraco-abdominal course. There is frequent involvement of visceral arterial branches such as renal and superior mesenteric arteries. Although the disease has been popularised by the appealing name of "abdominal coarctation", the term mid-aortic dysplastic syndrome is more appropriate. In spite of the fact that the syndrome was described almost three decades ago, its exact aetiology remains obscure and pathogenesis speculative. Surgical revascularisation remains the only therapeutic remedy, in dealing with this particular group of young hypertensive patients. The clinical presentation, angiographic assessment and long-term outcome, following reconstruction in 11 patients (mean age 24.4 years) with mid-aortic dysplastic syndrome were evaluated, in an effort to determine the effectiveness of surgery. Late follow-up, slightly exceeding 16 years (mean 5.6 years) has shown normal and relief from hypertension in practically all individuals. Furthermore, in order to elucidate at least some aspects of histopathology, studies were undertaken upon specimens from the aortic wall and the renal, carotid, lumbar and brachial arteries. The results suggested predominantly dysplastic features in the media, intima and particularly along the course of internal lamina. Aortography, using different projections, revealed variable patterns of high mid-aortic stenosis with or without associated renal artery disease. All patients were hypertensive (mean blood pressure 170 mmHg) and co-exist renal artery disease, unilateral in three and bilateral in three cases, was detected.(ABSTRACT TRUNCATED AT 250 WORDS)
 ...
PMID:The mid-aortic dysplastic syndrome. Surgical considerations with a 2 to 18 year follow-up and selective histopathological study. 232 23

Data from 35 patients with supravalvular aortic stenosis or pulmonary artery stenosis, or both, undergoing cardiac catheterization between 1973 and 1989 were analyzed retrospectively. Twenty-seven patients had supravalvular aortic stenosis: 11 required surgery after the first investigation and 8 (80%) of 10 others undergoing serial investigation showed an increase in the left ventricle to aorta pressure gradient. Angiographic measurements showed that the increase in the aortic pressure gradient was related to failure of normal growth of the ascending aorta lumen. Nineteen patients had pulmonary artery stenosis, with a right ventricular pressure greater than 33 mm Hg. At restudy, right ventricular pressure had decreased in 9 (82%) of 11 patients. This decrease in right ventricular pressure was associated with an increase in the systolic distensibility of the proximal pulmonary arteries, although there was no increase in the diastolic diameters. One patient had a rapid early increase in right ventricular pressure and no pulmonary artery growth. In two patients, multiple peripheral pulmonary artery stenoses became evident with time and produced persistent right ventricular hypertension. Supravalvular aortic stenosis is usually a progressive lesion, with an increase in left ventricular outflow tract pressure gradient related to poor growth of the ascending aorta. Pulmonary artery stenosis usually improves and only rarely limits prognosis.
 ...
PMID:Natural history of supravalvular aortic stenosis and pulmonary artery stenosis. 234 45

Central nervous system is rarely involved in progressive systemic sclerosis (PSS) unless there are concomitant abnormalities in renal or lung function or hypertension. A 72-year-old woman with typical PSS developed cerebellar bleeding. Medical history records revealed, she had noted the onset of Raynaud's sign on her upper extremities at the age of 37. This was followed by necrosis and repeated infection, and as a result, shortening of her fingers in her 40's. The disease progressed and involved lower extremities, and then face and body in her 50's. Aortic valve stenosis was diagnosed at 69 year old, cardiac myopathy at 70 and at the age of 71 infectious dermatitis in both inguinal regions. Mild anemia, hypoalbuminemia and the decrease of serum Fe were discovered in June 1988. At the same time, prolonged ESR, positive C-reactive protein, RA, and anti-nuclear-antibody were also noticed. A chest roentgenogram revealed pulmonary fibrosis. Systemic hypertension was not noticed on the clinical course. She developed an onset of vertigo and vomiting in the morning of August 8, 1988. Consequently, she was brought to our hospital. She was alert but a physical examination showed a swallowing disturbance, dysarthria, right cerebellar ataxia, nystagmus and hypertension (192/100 mmHg). A CT examination on admission revealed a slightly low density area in right cerebellar hemisphere without mass effect. She was treated with dextran and mannitol and her condition improved on the 6th day of her admission. She was alert and blood pressure calm down to 120/70 mmHg without the use of anti-hypertension drugs on August 21.(ABSTRACT TRUNCATED AT 250 WORDS)
 ...
PMID:[A case of progressive systemic sclerosis associated with a hemorrhagic infarction of the cerebellum]. 235 21


<< Previous 1 2 3 4 5 6 7 8 9 10 Next >>