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Hypertension Causes

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34741. BMPR2 Haploinsufficiency as the Inherited Molecular Mechanism for Primary Pulmonary Hypertension (PubMed)

BMPR2 Haploinsufficiency as the Inherited Molecular Mechanism for Primary Pulmonary Hypertension Primary pulmonary hypertension (PPH) is a potentially lethal disorder, because the elevation of the pulmonary arterial pressure may result in right-heart failure. Histologically, the disorder is characterized by proliferation of pulmonary-artery smooth muscle and endothelial cells, by intimal hyperplasia, and by in situ thrombus formation. Heterozygous mutations within the bone morphogenetic protein (...) and between families. Taken together, these studies illustrate the considerable heterogeneity of BMPR2 mutations that cause PPH, and they strongly suggest that additional factors, genetic and/or environmental, may be required for the development of the clinical phenotype.

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2000 American Journal of Human Genetics

34742. Sporadic primary pulmonary hypertension is associated with germline mutations of the gene encoding BMPR-II, a receptor member of the TGF-β family (PubMed)

Sporadic primary pulmonary hypertension is associated with germline mutations of the gene encoding BMPR-II, a receptor member of the TGF-β family Primary pulmonary hypertension (PPH), resulting from occlusion of small pulmonary arteries, is a devastating condition. Mutations of the bone morphogenetic protein receptor type II gene (BMPR2), a component of the transforming growth factor beta (TGF-beta) family which plays a key role in cell growth, have recently been identified as causing familial (...) PPH. We have searched for BMPR2 gene mutations in sporadic PPH patients to determine whether the same genetic defect underlies the more common form of the disorder.We investigated 50 unrelated patients, with a clinical diagnosis of PPH and no identifiable family history of pulmonary hypertension, by direct sequencing of the entire coding region and intron/exon boundaries of the BMPR2 gene. DNA from available parent pairs (n=5) was used to assess the occurrence of spontaneous (de novo) mutations

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2000 Journal of Medical Genetics

34743. A novel genetic locus for low renin hypertension: familial hyperaldosteronism type II maps to chromosome 7 (7p22) (PubMed)

A novel genetic locus for low renin hypertension: familial hyperaldosteronism type II maps to chromosome 7 (7p22) Familial hyperaldosteronism type II (FH-II) is caused by adrenocortical hyperplasia or aldosteronoma or both and is frequently transmitted in an autosomal dominant fashion. Unlike FH type I (FH-I), which results from fusion of the CYP11B1 and CYP11B2 genes, hyperaldosteronism in FH-II is not glucocorticoid remediable. A large family with FH-II was used for a genome wide search

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2000 Journal of Medical Genetics

34744. Primary pulmonary hypertension: the pressure rises for a gene (PubMed)

Primary pulmonary hypertension: the pressure rises for a gene Primary pulmonary hypertension (PPH) represents the end stage of a disruption of pulmonary vascular integrity, of unknown cause. Although PPH is associated with several systemic disorders, there have hitherto been few clues as to the aetiological factors responsible for the pathogenesis of this condition. As an example of the application of modern molecular genetics and positional cloning, this leader describes the range of studies

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2000 Journal of Clinical Pathology

34745. Acute and chronic effects of surgical thromboendarterectomy on exercise capacity and ventilatory efficiency in patients with chronic thromboembolic pulmonary hypertension (PubMed)

Acute and chronic effects of surgical thromboendarterectomy on exercise capacity and ventilatory efficiency in patients with chronic thromboembolic pulmonary hypertension To assess acute and chronic effects of surgical thromboendarterectomy on exercise capacity and ventilatory efficiency in patients with chronic thromboembolic pulmonary hypertension (CTEPH).Cardiopulmonary exercise testing was performed in 20 patients with CTEPH before thromboendarterectomy (baseline), one month after (early (...) ).Thromboendarterectomy may cause an immediate improvement in ventilatory efficiency, possibly through its beneficial haemodynamic effects. In contrast, exercise capacity may continue to improve towards the late phase, reflecting peripheral adaptation to exercise.

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2001 Heart

34746. Raised urinary glucocorticoid and adrenal androgen precursors in the urine of young hypertensive patients: possible evidence for partial glucocorticoid resistance (PubMed)

Raised urinary glucocorticoid and adrenal androgen precursors in the urine of young hypertensive patients: possible evidence for partial glucocorticoid resistance To evaluate urinary glucocorticoid excretion profiles in a cohort of recently diagnosed young hypertensive patients.After excluding patients with secondary causes, 60 individuals with premature hypertension were recruited (diagnosed by ambulatory blood pressure monitoring before the age of 36 years). In addition, 30 older hypertensive (...) controls (age of onset > 36 years, "middle aged hypertensive controls"), and 30 normal controls (age matched to the young hypertensive group) were studied. All provided 24 hour urine collections for mass spectrometry for total cortisol metabolites and total androgen metabolites by gas chromatography.Among male patients, those with premature hypertension had higher total urinary excretion of cortisol metabolites (mean (SD), 13 332 (6472) microg/day) than age matched normal controls (7270 (1788) microg

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2001 Heart

34747. Acute hemodynamic effects of inhaled nitric oxide, dobutamine and a combination of the two in patients with mild to moderate secondary pulmonary hypertension (PubMed)

Acute hemodynamic effects of inhaled nitric oxide, dobutamine and a combination of the two in patients with mild to moderate secondary pulmonary hypertension The use of low-dose dobutamine to maintain hemodynamic stability in pulmonary hypertension may have a detrimental effect on gas exchange. The aim of this study was to investigate whether inhaled nitric oxide (INO), dobutamine and a combination of the two have beneficial effects in patients with end-stage airway lung disease and pulmonary (...) hypertension.Hemodynamic evaluation was assessed 10 min after the administration of each drug and of their combination, in 28 candidates for lung transplantation.Administration of INO caused a reduction in mean pulmonary arterial pressure (MPAP), an increase in PaO2 with a significant reduction in venous admixture effect (Qs/Qt).Dobutamine administration caused an increase in cardiac index and MPAP, with a decrease in PaO2 as a result of a higher Qs/Qt. Administration of a combination of the two drugs caused

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2001 Critical Care

34748. Idiopathic non-cirrhotic intrahepatic portal hypertension in the West: a re-evaluation in 28 patients (PubMed)

Idiopathic non-cirrhotic intrahepatic portal hypertension in the West: a re-evaluation in 28 patients Non-cirrhotic portal hypertension of unknown cause is a poorly understood condition attributed to obstructive portal venopathy.To reassess the manifestations, course, and causes, with special attention to thrombosis.Analysis of a cohort of 28 patients.Gastrointestinal bleeding occurred in 11 patients. Liver failure developed at the time of concurrent disease in eight patients, including all

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2002 Gut

34749. Diuretic treatment of resistant hypertension. (PubMed)

or extracellular fluid volume is an important cause of resistance to treatment even when a thiazide diuretic is used. An increase in diuretic treatment should be tried before using the postganglionic adrenergic blockers or minoxidil in resistant hypertension. (...) Diuretic treatment of resistant hypertension. In patients with hypertension resistant to three or four drugs including a thiazide diuretic substitution of frusemide for the thiazide, or the addition of spironolactone, produced significant reductions in blood pressure and body weight. The response did not depend on the presence of overt fluid retention, renal impairment, or the use of antihypertensive drugs of high potency. Women had larger responses than men. Expansion of the plasma

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1980 British medical journal

34750. Infarction of the optic nerve head in children with accelerated hypertension. (PubMed)

Infarction of the optic nerve head in children with accelerated hypertension. Four cases of anterior ischaemic optic neuropathy occurred in children with accelerated hypertension. The cause may have been a sudden relative fall in arterial pressure which reduced the perfusion of the optic disc, whose circulation was compromised by long-standing hypertensive vascular disease.

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1981 The British journal of ophthalmology

34751. Portal diversion for portal hypertension in children. The first ninety patients. (PubMed)

Portal diversion for portal hypertension in children. The first ninety patients. Ninety children with portal hypertension were treated by portal diversion. Fifty-two had cavernous transformation of the portal vein and 38 had an intrahepatic block from various causes. There were 59 central splenorenal shunts, 19 mesocaval, 11 portacaval and one distal splenorenal. In 61 peripheral shunts the veins used for the anastomosis were less than 10 mm in diameter. There was no operative mortality (...) in the management of portal hypertension in childhood and suggest that portal diversion is the treatment of choice. Several precautions have permitted lowering of the rate of thrombosis whichever shunt is performed. Portal diversion should be indicated following the first episode of hemorrhage in children with extrahepatic block. In patients with intrahepatic block, congenital hepatic fibrosis and cystic fibrosis are good indications as are in general the hepatic diseases with no or mild activity.

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1980 Annals of Surgery

34752. Upper airways obstruction. Presentation with systemic hypertension. (PubMed)

Upper airways obstruction. Presentation with systemic hypertension. Of 14 patients whose final diagnosis was upper airways obstruction associated with heart failure, 3 presented with systemic hypertension (up to 200/100 mmHg). In 2 the hypertension was so severe that at first it had to be considered as a possible cause of the presenting symptoms. The subsequent history indicated that it was an effect of the upper airways obstruction with heart failure.

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1981 Archives of Disease in Childhood

34753. Significance of corrected sinusoidal pressure (CSP) in patients with cirrhosis and portal hypertension. (PubMed)

in establishing extrahepatic or presinusoidal causes of portal hypertension. (...) Significance of corrected sinusoidal pressure (CSP) in patients with cirrhosis and portal hypertension. The relationship between the degree of portal hypertension measured by the corrected sinusoidal pressure (CSP), the size of varices graded endoscopically, the risk of variceal hemorrhage and the prognosis following portal decompression were studied in 32 patients with cirrhosis demonstrated by examination of a biopsy specimen. The CSP was no different in patients with large-sized versus small

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1981 Annals of Surgery

34754. Hemodynamic guidelines for surgical therapy of portal hypertension. (PubMed)

Hemodynamic guidelines for surgical therapy of portal hypertension. A prospective study of 98 patients with portal hypertension who hemorrhaged revealed that certain hemodynamic parameters were valuable in confirming the cause of cirrhosis, aiding in the selection of patients best suited for a selective distal splenorenal shunt, and in providing an estimate of prognosis. The presence of a pressure gradient of 4 mmHg or more between the right atrium and inferior vena cava was observed only (...) in patients with alcoholic cirrhosis. The shape of the "pull-back" tracing between the wedge and free hepatic vein positions was "smooth" in postnecrotic disease and "lumpy" in alcoholic disease. The ratio of the aortic diastolic pressure divided by the hepatic (vein) wedge pressure segregated patients by cause and direction of portal blood flow.

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1981 Annals of Surgery

34755. Disseminated Intravascular Coagulation and Portal Hypertension Following Pancreatic Islet Autotransplantation (PubMed)

Disseminated Intravascular Coagulation and Portal Hypertension Following Pancreatic Islet Autotransplantation A patient undergoing subtotal pancreatectomy and intraportal islet tissue autotransplantation for chronic pancreatitis developed severe portal hypertension (49 cm of H(2)O) and acute disseminated intravascular coagulation (DIC). In an attempt to identify the cause of these problems, portal pressure and the activities of the coagulation and fibrinolytic systems were studied in dogs

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1980 Annals of Surgery

34756. Primary pulmonary hypertension: effects of nifedipine. (PubMed)

Primary pulmonary hypertension: effects of nifedipine. The results of the medical treatment of primary pulmonary hypertension are usually very poor. A case of severe primary pulmonary hypertension in chronic heart failure was treated with the calcium antagonist nifedipine, a powerful vasodilator which acts directly on vascular smooth muscle. Nifedipine, administered in a dose of 20 mg sublingually, caused a pronounced fall in pulmonary and systemic vascular resistances (54 and 49%, respectively

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1980 British Heart Journal

34757. Effect of heme arginate administration on blood pressure in spontaneously hypertensive rats. (PubMed)

/omega-1 hydroxylases leading to a marked decrease in 19-hydroxyeicosatetraenoic acid (HETE) and 20-HETE. The effect of heme arginate on blood pressure may be mediated via these biochemical events inasmuch as both 19-HETE and 20-HETE produced by the kidney may promote hypertension by causing vasoconstriction and sodium retention. (...) Effect of heme arginate administration on blood pressure in spontaneously hypertensive rats. Cytochrome P450 content and activities are increased in the kidneys of spontaneously hypertensive rats (SHR) as compared with those of normotensive, Wistar-Kyoto (WKY), control rats during the period of rapid elevation of blood pressure. We studied the effect of heme arginate, a potent inducer of heme oxygenase (EC 1.14.99.3), on microsomal cytochrome P450 levels and activities and blood pressure in SHR

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1990 Journal of Clinical Investigation

34758. Red cell membrane sodium transport: possible genetic role and use in identifying patients at risk of essential hypertension. (PubMed)

hypertensive children were excluded and the normotensive siblings with and without hypertensive family histories were compared. These data suggest that abnormal red cell membrane sodium transport has a familial component, and although it is not caused by the hypertension it may be the earliest pathophysiological step in its development, perhaps allowing the identification of children at risk of essential hypertension. (...) Red cell membrane sodium transport: possible genetic role and use in identifying patients at risk of essential hypertension. To investigate the influence of a family history of essential hypertension on abnormalities of red cell membrane sodium transport, 28 hypertensive children and their families were studied. In 15 families one or both parents had either essential hypertension or a strong family history. In 13 families neither parent had essential hypertension or a positive family history

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1990 Archives of Disease in Childhood

34759. Essential hypertension: a sign in search of a disease. (PubMed)

Essential hypertension: a sign in search of a disease. The concept of disease is of cardinal importance in medical practice. The current definition has developed over more than 200 years. It includes a distinctive natural history and identifiable cellular changes. Pickering proposed a fundamental alteration to the definition when he suggested that essential hypertension is a quantitative disease without causative cellular change distinguishing normal from abnormal. The nature of essential (...) hypertension has been confused from the beginning because of a category error. Injury is conceptually distinguished from disease. Essential hypertension, defined as elevated blood pressure together with its cardiovascular consequences, is found to be neither an injury nor a disease according to current definitions. Instead, essential hypertension refers to a treatment group just as "the fevers" did in an earlier century. One effect on patients of the failure to resolve this diagnostic paradox is the burden

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1991 CMAJ: Canadian Medical Association Journal

34760. Hydatid Cysts of Liver and Portal Hypertension (PubMed)

Hydatid Cysts of Liver and Portal Hypertension Two cases of portal hypertension due to hydatid cysts of the liver are reported. In one of the patients, symptoms were secondary to obstruction of inferior vena cava and hepatic outflow tract. The other patient was operated on with a diagnosis of extrahepatic presinusoidal portal hypertension caused by extrinsic compression of the liver by an hydatid cyst. Although hydatidosis is a benign disease, it can produce serious complications (...) as in these reported cases. Therefore hydatidosis should be remembered amongst the causes of portal hypertension in countries where the disease is endemic.

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1990 HPB Surgery

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