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

Primary hyperaldosteronism (PHA) is regarded as a rare disease with prevalence rates of 0.5 to 2% within the hypertensive population. Recent studies using more detailed screening procedures in small hypertensive cohorts have suggested that PHA may be more common than previously thought (3-18%). Since a validated and cost-effective routine screening protocol for this entity is not established, many clinicians are reluctant to consider PHA as an underlying cause for a patient's high blood pressure. The insufficient perception of PHA may have fatal consequences since most patients are curable by an operation and missing the diagnosis often leads to significant and irreversible end-organ damage. This review focuses on the diagnosis of PHA and gives a rational and cost-effective flow chart for routine screening and differential diagnosis of PHA in hypertensive patients.
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PMID:Primary hyperaldosteronism. 1237 29

Retroperitoneal fibrosis is an inflammatory disease, which is either idiopathic or secondary to infection, neoplasm, hemorrhage, aortic aneurysm or drugs. This is a rare disease usually presenting constitutional symptoms, abdominal, back or flank pain and urinary frequency. Treatment includes surgical relief of urethral obstruction and corticosteroids. There is no clear evidence of the beneficial effect of corticosteroids treatment on the course of retroperitoneal fibrosis. We report a patient diagnosed with retroperitoneal fibrosis with an unusual presentation--uncontrolled HTN and renal failure due to renal arteries obstruction, without any abdominal symptoms. This patient responded to steroids and tamoxifen. A review of the literature is also presented.
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PMID:[Retroperitoneal fibrosis--clinical response to steroid treatment]. 1269 65

Spontaneous dissecting aneurysm of the coronary artery (SDACA) not associated with aortic aneurysm or trauma is a very rare cause of myocardial ischemia. It has a higher prevalence in otherwise healthy women, especially in the peri- and postpartum period. In 80-90% of cases the diagnosis of SDACA is confirmed at autopsy, as the majority of patients present with acute myocardial infarction or sudden death. We describe the case of a 47-year-old Caucasian woman who died suddenly without any previous sign of cardiac disease. She had no clinical signs of Marfan's syndrome or arterial hypertension, she used no oral contraceptives and she was not in the peri- or postpartum period. At autopsy we found a recent dissecting aneurysm of the right coronary artery. Histology showed a hematoma between the media and adventitia of the coronary artery, flattening and occluding the lumen. Acute myocardial infarction was present in the posterior wall of the left cardiac ventricle. SDACA is an unpredictable condition and prompt diagnosis and life-saving procedures--either surgical or conservative--are rarely successful. We report a very rare disease documented in only 108 cases according to the English-language literature accessible to us.
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PMID:Spontaneous dissecting aneurysm of the coronary artery. A rare cause of myocardial infarction and sudden death. 1280 Apr 49

Idiopathic infantile arterial calcification (IIAC) is a rare disease that is characterized by calcification in the media and fibroproliferative changes in the intima of larger arteries, sometimes resulting in reduced vascular elasticity and blood flow. Although the molecular-genetic basis of the disease is unknown, IIAC is presumed to be acquired by an autosomal recessive mode of inheritance and is associated with a reduction in the levels of enzymes responsible for inorganic phosphate balance, resulting in abnormal deposition of calcium into the vessels. We report the case of a female neonate in whom widespread IIAC was initially diagnosed on postnatal sonographic examination. At birth, the infant experienced cardiac failure and hypertension, and arterial pulsation was absent. Routine prenatal sonographic examinations had not revealed any abnormalities, but postnatal gray-scale and color Doppler echocardiographic and sonographic examinations revealed findings consistent with severe IIAC. The cardiac function improved with treatment, but the neonate died of progressive hepatic failure due to reduced flow in calcified and narrowed hepatic arteries. The common carotid arteries were also grossly affected, resulting in cerebral atrophy at the time of birth. Postnatal gray-scale and color Doppler echocardiographic and sonographic examinations allowed noninvasive diagnosis, assessment of severity, and monitoring of progression.
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PMID:Idiopathic infantile arterial calcification: sonographic findings. 1459 43

Idiopathic spontaneous renal artery dissection (SRAD) is a rare disease and must be taken into account in the differential diagnosis of low back pain. It may be due to various aetiologies, secondary to degenerative or traumatic diseases, or it may be idiopathic. Intravenous urography is usually normal. Abdominal CT usually visualizes the renal infarction and selective arteriography confirms the diagnosis of renal artery dissection. Medical treatment and surveillance provide effective management of the disease. However, surgical management may be proposed either immediately or secondarily. SRAD usually has a favourable course, but, in the longer term, may be complicated by organic renal failure and renovascular hypertension.
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PMID:[Partial infarction of the kidney caused by spontaneous idiopathic dissection of the renal artery]. 1465 Mar 2

Obesity is closely associated with the Metabolic Syndrome, which includes insulin resistance, glucose intolerance, dyslipidemia and hypertension. The best predictor of these morbidities is not the total body fat mass but the quantity of visceral (e.g. omental, mesenteric) fat. Glucocorticoids play a pivotal role in regulating fat metabolism, function and distribution. Indeed, patients with Cushing-s syndrome (a rare disease characterized by systemic glucocorticoid excess originating from the adrenal or pituitary tumors) or receiving glucocorticoid therapy develop reversible visceral fat obesity. The role of glucocorticoids in prevalent forms of human obesity, however, has remained obscure, because circulating glucocorticoid concentrations are not elevated in the majority of obese subjects. Glucocorticoid action on target tissue depends not only on circulating levels but also on intracellular concentration. Locally enhanced action of gluccorticoids in adipose tissue and skeletal muscle has been demonstrated in the Metabolic Syndrome. Evidence has accumulated that enzyme activity of 11beta-hydroxysteroid dehydrogenase type 1 (11beta-HSD1), which regenerates active glucocorticoids from inactive forms and plays a central role in regulating intracellular glucocorticoid concentration, is commonly elevated in fat depots from obese individuals. This suggests a role for local glucocorticoid reactivation in obesity and the Metabolic Syndrome. 11beta-HSD1 knockout mice resist visceral fat accumulation and insulin resistance even on a high-fat diet. Furthermore, fat-specific 11beta-HSD1 transgenic mice, those have increased enzyme activity to a similar extent seen in obese humans, develop visceral obesity with insulin and leptin resistance, dyslipidemia and hypertension. In adipocytes, both antidiabetic PPARgamma agonists and LXRalpha agonists significantly reduce 11beta-HSD1 mRNA and enzyme activity, suggesting that suppression of 11beta-HSD1 in adipose tissue may be one of the mechanisms by which these drugs exert beneficial metabolic effects. Recently reported selective inhibitors of 11beta-HSD1 can ameliorate severe hyperglycemia in the genetically diabetic obese mice. In summary, 11beta-HSD1 is a promising pharmaceutical target for the treatment of the Metabolic Syndrome.
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PMID:Tissue-specific glucocorticoid reactivating enzyme, 11 beta-hydroxysteroid dehydrogenase type 1 (11 beta-HSD1)--a promising drug target for the treatment of metabolic syndrome. 1468 56

CLASSICAL FEATURES AND SCREENING: The classical features of primary aldosteronism-hypertension, hypokalemia and metabolic alkalosis-were first described by J. Conn in the midfifties of the last century. The classical form of primary aldosteronism is a rare disease with prevalence rates of 0.1-0.5% within the hypertensive population. The normokalemic variant of primary aldosteronism seems to be much more frequent (5-13%). Although a validated and standardized diagnostic protocol for this entity is still missing recent studies established the aldosterone to renin ratio as a useful screening test. To increase diagnostic sensitivity and specificity of the ratio aldosterone should be added as second screening criterion (sensitivity and specificity about 90%). Dynamic confirmatory testing proving autonomous aldosterone secretion is required to verify the diagnosis in case of a positive screening test. A simple confirmatory test is the salt loading test. Alternatively, the fludrocortisone-suppression-test, the Captopril-challenge- test or the daily exretion rate of aldosterone-18-glucuronide and tetrahydroaldosterone in urine can be used. In case of proven primary aldosteronism further diagnostic evaluation (e. g. CT scanning, postural-test and in case of discrepancy adrenal vein catheterization) is mandatory to differentiate the most common forms of primary aldosteronism, aldosterone producing adenoma and idiopathic hyperaldosteronism. Since many patients with primary aldosteronism can be cured by surgery and missing the diagnosis often leads to significant end-organ damage it is important to evaluate hypertensive patients with therapy-resistant hypertension for primary aldosteronism.
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PMID:[The aldosterone to Renin ratio in secondary hypertension]. 1468 2

Primary pulmonary hypertension is a rare disease of the pulmonary vasculature manifested by dyspnea on exertion, syncope, and signs and symptoms of right heart failure. In the absence of adequate treatment, primary pulmonary hypertension has a grave prognosis, with a median survival of 2.8 years. Pulmonary arterial hypertension develops in association with known risk factors and predisposing clinical conditions, and shares many clinical, pathological and therapeutic characteristics with primary pulmonary hypertension. Therapeutic choices in pulmonary arterial hypertension depend on the etiology of the disease, severity of functional impairment and hemodynamic response following acute vasodilator administration during right heart catheterization. Agents currently approved for the specific treatment of pulmonary arterial hypertension are continuous intravenous epoprostenol, subcutaneous treprostinil and oral bosentan. A small group of patients who demonstrate true acute vasoreactivity at right heart catheterization may be chronically treated with oral calcium channel blockers. In addition, most patients with pulmonary hypertension receive conventional treatment, represented by anticoagulants, diuretics, inotropic medication or oxygen supplementation. Treatment of pulmonary arterial hypertension has significantly altered the natural course of the disease, with pronounced symptomatic, functional and survival benefit. Current clinical research focuses on the discovery of new targets of therapy and the use of a combination treatment approach, which will offer hope and valuable insight into the pathogenetic basis of this devastating illness.
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PMID:Current medical treatment of pulmonary arterial hypertension. 1502 2

Pulmonary hypertension (PH) is a rare disease with a very poor prognosis. Certain pharmacologic approaches, which reduce pulmonary arterial pressure (PAP) and thereby prevent end-stage cardiopulmonary failure, have been used during recent years. Endothelin-1 has been found to be involved in the pathogenesis of PH. The dual endothelin-receptor antagonist, bosentan, was recently approved for the treatment of pulmonary arterial hypertension. The drug is mainly cleared by hepatic elimination. Severe renal dysfunction does not affect the single-dose pharmacokinetics of bosentan to a clinically relevant extent. Whether renal replacement therapy, however, interferes with the pharmacokinetics of bosentan is unknown. The authors report on the use of bosentan (125 mg twice daily) and its pharmacokinetic monitoring in a 19-year-old woman with PH and end-stage renal disease secondary to scleroderma. Treatment was well tolerated without drug-specific adverse effects. After 12 months of treatment, pulmonary arterial pressure had normalized (48 mm Hg before start of treatment, 27 mm Hg at last follow-up). On the basis of analyzing samples from Genius-hemodialysis by a liquid chromatography assay with tandem mass spectrometry detection, the authors determined the bosentan dialysis clearance to be as low as 3.5 mL/min. Bosentan for the treatment of secondary PH seems to be safe as well as effective in end-stage renal disease patients and no adjustment of the bosentan dosing regimen appears necessary.
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PMID:Treatment of secondary pulmonary hypertension with bosentan and its pharmacokinetic monitoring in ESRD. 1511 84

Renal artery aneurysm is a rare disease in children and usually is due to fibromuscular dysplasia. Clinical symptoms are frequently high blood pressure, abdominal pain, and hematuria. Diagnosis is carried out by means of angiography. We report the case of a 13-year-old male patient who had renovascular hypertension due to calcification and aneurysm of fibromuscular dysplasia-associated renal artery. We carried out total nephrectomy to resolve high blood pressure. We suggested that presence of discreet calcification in region of renal artery in a boy with renovascular high blood pressure should guide us toward diagnosis of fibromuscular dysplasia-related renal artery aneurysm.
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PMID:[Calcified renal artery aneurism and high blood pressure. A case report and review of the literature]. 1531 Apr 49


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