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

Portopulmonary hypertension is a rare complication of portal hypertension. Although epoprostenol infusion, nitric oxide inhalation, isosorbide-5-mononitrate, nitroglycerin, and calcium channel blockers may reduce pulmonary artery pressure in patients with portopulmonary hypertension, the prognosis remains poor. We present a case of congenital hepatic fibrosis associated with pulmonary hypertension. A 42-year-old man with congenital hepatic fibrosis visited our hospital with syncope. The man had suffered from breathlessness on exertion for 2 weeks before the episode of syncope. He also had a history of portal hypertension with documented gastric cardiac varices at the age of 28 years. Despite undergoing intensive care, the patient died 1 week after admission owing to severe right-sided heart failure. Autopsy revealed dilatation of the right atrium and right ventricle grossly and plexogenic pulmonary arteriopathy microscopically. Accurate diagnosis of portopulmonary hypertension requires awareness of the disease and a high index of suspicion when examining patients with portal hypertension and dyspnea.
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PMID:Severe portopulmonary hypertension in congenital hepatic fibrosis. 1279 Feb 24

Our aim was to study the cause and describe the clinical features of pulmonary arterial hypertension (PHT) in splenectomized beta-thalassemia (beta-Thal) patients. Ten splenectomized beta-Thal patients with systolic pulmonary artery (PA) pressure >30 mm Hg were evaluated by echocardiography, right-heart catheterization, and pulmonary angiography. Five of these patients later underwent hemodynamic studies. Echocardiography and pulmonary angiography on the 10 patients showed normal values of left ventricular systolic function and no findings of acute or chronic pulmonary embolism. Hemodynamic evaluation showed very high PA pressures associated with markedly increased pulmonary vascular resistance indices (PVRIs). Hematological evaluation of the 10 patients showed marked anemia, markedly increased numbers of nucleated red blood cells (nRBCs), and serum ferritin. Mean platelet count, plasma beta2 thromboglobulin, and thrombin-antithrombin III complex levels were significantly increased. It was concluded that PHT can be found in splenectomized beta-Thal patients. Features associated with PHT were female sex, hemoglobin E/beta-Thal, status many years postsplenectomy, marked anemia, markedly increased nRBC count, thrombocytosis, and very high serum ferritin levels. PHT was not due to pulmonary emboli. Our findings suggested that severe PHT was due to increased PVRI from thrombotic pulmonary arteriopathy, likely from chronic low-grade hypercoagulability and platelet activation after splenectomy.
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PMID:Pulmonary arterial hypertension in previously splenectomized patients with beta-thalassemic disorders. 1295 8

Fibromuscular dysplasia is a multifactorial arteriopathy that primarily affects small and medium-sized arteries. It is most common in the renal and internal carotid arteries. Pathological classification is based on the arterial wall layer most significantly involved. The natural history and incidence of asymptomatic disease is unknown. The most common lesions become symptomatic as a high-grade stenosis producing renovascular hypertension or as an embolic source for the cerebral circulation. Treatment is reserved for symptomatic lesions. Most simple lesions are effectively treated by catheter-based intervention. Surgical therapy is warranted for more complex lesions. Both produce durable, long-term results.
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PMID:Fibromuscular dysplasia: when is intervention warranted? 1297 58

Retinal vein occlusion (RVO) is a relatively common disease, often associated with the presence of diseases related to internal medicine. It is well known that RVO is associated with common systemic vascular disorders such as hypertension, arteriosclerosis and diabetes. Several studies using hospital-based controls have shown an increased risk of RVO in patients with arteriopathy, or high levels of plasma glucose and arterial blood pressure. Patients are categorized into six types of RVO based on the site of occlusion and on the type of consequent vascular damage. Central retinal vein occlusion (CRVO) is the most frequently-occurring and clinically relevant type of RVO. In addition to the well-known classical risk factors, new haemostasis-related ones have been investigated in patients affected by CRVO. While data concerning a number of parameters remain contradictory, high levels of type 1 plasminogen activator inhibitor (PAI-1) and hyperhomocysteinemia appear to play a significant role in the pathogenesis of this disease. Although based on a limited number of studies, this new knowledge could eventually provide important indications regarding prognosis and therapeutic strategies. There is no established treatment for CRVO. Treatment consists primarily of managing any identified underlying systemic disease. The increasing role of hypercoagulability in patients with CRVO supports the use of antithrombotic drugs in the treatment of this disease. Vitamin treatment to correct hyperhomocysteinemia should also be taken into consideration. However, the approach to CRVO treatment with antithrombotic drugs is not evidence-based yet. There is urgent need of intervention trials to evaluate the role of these drugs in CRVO patients.
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PMID:Retinal vein thrombosis: risk factors, pathogenesis and therapeutic approach. 1367 63

The association of membranous glomerulonephritis with benign tumors is rarely described. This report represents, to the best of our knowledge, the first documented account of the simultaneous occurrence of juxtaglomerular cell tumor of the kidney and membranous glomerulonephritis. A young Chinese woman presented with hypertension and proteinuria, whereupon investigations disclosed a renal tumor. She underwent surgical resection, and histologic examination of the tumor revealed CD34-positive uniform polygonal cells with accompanying mast cells, disposed in sheets with focal papillary pattern punctuated by cytokeratin-positive tubular structures. Characteristic rhomboid crystalline granules were identified ultrastructurally. The kidney adjacent to the tumor showed features of membranous glomerulonephritis and hypertensive arteriopathy. Proteinuria improved following tumor resection, but the patient had persistent hypertension attributable to renal hypertensive arteriopathy. This report also highlights the recently described observation of CD34 positivity in juxtaglomerular cell tumors.
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PMID:A case of juxtaglomerular cell tumor associated with membranous glomerulonephritis. 1457 36

Chronic transfusion of packed red blood cells, in addition to other ongoing treatment with warfarin, acetyl salicylic acid, desferrioxamine, and other supportive measures, was given to a splenectomized hemoglobin E/beta-thalassemia woman with pulmonary arterial hypertension (PHT). Serial measurements of plasma thrombin-antithrombin III complex (TAT) levels and right-sided cardiac catheterization were used to monitor changes after treatment. Reduction of plasma TAT levels from 7.5 to 3.8 microg/L (normal, 3 +/- 2.4 microg/L), pulmonary vascular resistance (PVR) from 553.8 to 238.6 dyne.sec.cm(-5) (normal, 67 +/- 30 dyne.sec.cm(-5)), and mean pulmonary arterial pressure from 51 to 32 mm Hg (normal, 9 to 19 mm Hg) occurred in tandem. Normalization of blood hypercoagulability as reflected in plasma TAT level by chronic blood transfusion was the likely basis for improvement of increased PVR, being secondary to thrombotic pulmonary arteriopathy and subsequently PHT.
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PMID:Correction of hypercoagulability and amelioration of pulmonary arterial hypertension by chronic blood transfusion in an asplenic hemoglobin E/beta-thalassemia patient. 1464

Understanding the features of the various hereditary vascular pathologies allows consideration and confirmation of the diagnosis, and a search for treatable hidden disorders, avoiding harmful investigations, initiating follow up, performing family investigations and providing genetic counselling. Pseudoxanthoma elasticum must be considered in the presence of calcified distal arteriopathy of the lower limbs in a young subject without any other aetiological aspects. Cutaneous or mucosal lesions confirmed on histological examination, angioid streaks at the back of the eye and a family history support the diagnosis, which is confirmed by showing pathogenic mutations of the ABCC6 gene. It is then important to search for a peripheral disorder in other arterial territory, a low grade coronaropathy, hypertension and an endocardial disorder. Prescription of antithrombotics must be made carefully because of the risk of gastro-intestinal haemorrhage. Vascular Ehlers-Danlos syndrome is suspected in a subject less than 30 years old with diffuse aneurysmal disease, spontaneous arterial rupture or dissection, a carotido-cavernous fistula or early onset varices. Demonstrating an ecchymotic tendency or an acrogeric morphology, especially in a familial context, warrants cutaneous biopsy for anatomopathological examination and fibroblast culture for a study of the C0L3A1 gene. When the diagnosis is suggested, it is advisable to prohibit any arterial puncture, cold surgery or gastro-intestinal endoscopy. The search for aneurysmal lesions must be performed by non-invasive imaging. The therapeutic management requires specialised teams. The combination of repeated epistaxis, muco-cutaneous telangectasia and similar characteristics in a family suggests the diagnosis of Osler-Weber-Rendu disease. The search for iron deficiency, gastro-intestinal bleeds and pulmonary, hepatic or cerebral arterio-venous malformations is then necessary. Besides skilled endovascular management when indicated, it is important to advise every patient with a pulmonary arterio-venous malformation to take antibiotic prophylaxis against cerebral abscess in situations at risk of bacteraemia.
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PMID:[Mendelian arterial diseases. Pseudoxanthoma elasticum, Ehlers-Danlos vascular syndrome, Rendu-Osler disease]. 1469 86

Ischemic damage to the subcortical white matter of the brain, referred to as leukoaraiosis, is a frequent complication of hypertension-related microvascular disease and contributes to the risk of stroke and vascular dementia. A large genetic contribution to this late-life form of target organ damage was suggested by a study of elderly male twins. As part of the Genetic Epidemiology Network of Arteriopathy (GENOA), 483 non-Hispanic white subjects were recruited to undergo MRI for determination of the brain volume of leukoaraiosis (291 women and 192 men from 210 sibships providing 434 sibling pairs; mean age+/-SD=65.2+/-7.3 years). The GENOA-Rochester sibships contain 2 or more siblings with essential hypertension diagnosed before age 60. The frequency distribution of the volume of leukoaraiosis was positively skewed, with a median value of 6.61 cm3 (interquartile range: 4.77 to 9 0.83 cm3). Variance component models were used to estimate the heritability (ie, the proportion of phenotypic variation caused by additive genetic factors). After logarithm transformation of the volume of leukoaraiosis, the estimated heritability (+/-SE) was 0.802+/-0.102 (P<0.0001). Adjustments for sex, age, systolic blood pressure, and brain volume reduced the heritability estimate to 0.671+/-0.110 (P<0.0001). This evidence of strong genetic influence on the susceptibility to leukoaraiosis justifies efforts to localize the responsible genes and characterize the predisposing genetic polymorphisms.
Hypertension 2004 Feb
PMID:Heritability of leukoaraiosis in hypertensive sibships. 1471 59

Few studies have investigated whether ethnic groups differ in the prevalence of peripheral arterial disease (PAD). We compared the distribution of the ankle-brachial index (ABI), a measure of PAD, between African Americans and non-Hispanic white individuals. Subjects (n = 931) belonged to the Genetic Epidemiology Network of Arteriopathy (GENOA) study, a community-based study of hypertensive sibships, and included 453 African Americans from Jackson, Mississipi (mean age 72 +/- 6 years, 69% women) and 478 non-Hispanic white individuals from Rochester, Minnesota (mean age 58 +/- 7 years, 64% women). ABI was determined at two sites in each lower extremity and the lowest of four indices was used in the analyses. PAD was defined as an ABI of < or = 0.95. Information about conventional risk factors was derived from interviews and from blood samples drawn at the study visit. The prevalence of diabetes and hypertension was significantly higher in African Americans than in non-Hispanic white individuals. After adjusting for age, African American subjects had a lower mean ABI (women 0.97 vs 1.04, p < 0.001; men 0.96 vs 1.12, p < 0.001) and a greater prevalence of PAD (women 34% vs 22%, p = 0.010; men 33% vs 11%, p < 0.001) than their non-Hispanic white counterparts. In multiple regression analyses, African American ethnicity was a predictor of a lower ABI and the presence of PAD in each sex after adjusting for age and other conventional risk factors. In conclusion, the lower ABI and greater prevalence of PAD in African Americans than in non-Hispanic white individuals is not explained by differences in conventional risk factors. Identifying additional 'novel' risk factors that account for the ethnic differences in PAD is an important next step towards understanding why such differences exist and developing more effective strategies to reduce the burden of PAD.
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PMID:Ethnic differences in peripheral arterial disease in the NHLBI Genetic Epidemiology Network of Arteriopathy (GENOA) study. 1512 83

Takayasu's arteritis is a rare idiopathic arteriopathy that produces narrowing and eventually closure of affected blood vessels. Ocular symptoms typically occur late in the disease process in those individuals who have severe involvement of the aortic arch and the carotid arteries. Visual loss typically occurs as a result of either systemic hypertension or concomitant ocular hypo-perfusion. We describe an unusual case of Takayasu's arteritis presenting in a child who complained of painful blurred vision. In this individual, no carotid artery disease was identified on vascular imaging and no evidence of impairment of ocular perfusion was demonstrable. Vision loss in this instance was due to anterior uveitis and cystoid maculopathy. These are atypical and rarely described features of Takayasu's arteritis. The patient responded well to anti-inflammatory treatment and his symptoms resolved. The clinical significance of these findings are discussed and the relevant literature reviewed.
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PMID:Anterior uveitis in a child with Takayasu's arteritis. 1518 Aug 52


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