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

Arterial stiffness has independent predictive value for cardiovascular events. We review data concerning the heritability of arterial stiffness, and propose an integrated view of the structural and genetic determinants of arterial stiffness, based on a candidate gene approach and recent studies on gene expression profile. Arterial stiffness seems to have a genetic component, which is largely independent of the influence of blood pressure and other cardiovascular risk factors. In animal models of essential hypertension (SHR and SHR-SP), structural modifications of the arterial wall include an increase in the number of elastin/smooth muscle cell (SMC) connections, and smaller fenestrations of the internal elastic lamina, possibly leading to redistribution of the mechanical load toward elastic materials. These modifications may give rise to mechanisms that explain why changes in arterial wall material accompanying wall hypertrophy in these animals are not associated with an increase in arterial stiffness. In monogenic connective tissue diseases (Marfan, Williams, and Ehlers-Danlos syndromes) and the corresponding animal models, precise characterization of the arterial phenotype makes it possible to determine the influence of abnormal genetically determined wall components on arterial stiffness. Such studies have highlighted the role of extracellular matrix signaling in the vascular wall and have shown that elastin and collagen not only display elasticity or rigidity but also are involved in the control of SMC function. These data provide strong evidence that arterial stiffness is affected by the amount and density of stiff wall material and the spatial organization of that material.
Hypertension 2005 Jun
PMID:Structural and genetic bases of arterial stiffness. 1643 22

The microfibrillar protein fibrillin-1 is a component of the mesangial matrix. Defects in fibrillin-1 predisposes individuals to vascular damage in Marfan syndrome, but the role of fibrillin-1 in kidney disease is unknown. We hypothesized that fibrillin-1 is involved in hypertensive or diabetic glomerular disease. DOCA-salt hypertension or streptozotocin (STZ) diabetes led to a significant increase in glomerular fibrillin-1 deposition. To test the functional role of fibrillin-1, DOCA hypertension and STZ diabetes were induced in mice homozygous for a mutation leading to a fivefold lower expression of fibrillin-1 (mgR/mgR). Untreated male mgR/mgR mice usually die from aortic dissection during the first 4 mo of life. All DOCA-treated mgR/mgR mice died within 2 wk after onset of DOCA treatment. DOCA-treated heterozygous (mgR/+) and their wild-type littermates displayed similar blood pressure levels, but albuminuria was significantly lower in mgR/+ than in wild-type mice after DOCA treatment. Similarly, STZ diabetic mgR/mgR and mgR/+ developed lower albuminuria than wild-type mice despite higher blood glucose levels in mgR/mgR and mgR/+ compared with wild-type mice. Blood pressure, blood glucose, and albuminuria did not differ among untreated mgR/mgR, mgR/+, and wild-type mice, respectively. In diabetic mgR/+ and mgR/mgR, but not in wild-type mice, an induction of glomerular decorin expression was observed. Thus underexpression of fibrillin-1 predisposes individuals to lethal aortic dissection in the presence of hypertension. On the other hand, albuminuria as a parameter of microvascular damage in hypertension and diabetes was ameliorated in fibrillin-1-underexpressing mice, possibly due to a compensatory upregulation of decorin. We conclude that fibrillin-1 may contribute to glomerular damage in hypertensive and diabetic kidney disease.
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PMID:Role of fibrillin-1 in hypertensive and diabetic glomerular disease. 1638 Apr 60

Aortic aneurysm and dissection are manifestations of Marfan syndrome (MFS), a disorder caused by mutations in the gene that encodes fibrillin-1. Selected manifestations of MFS reflect excessive signaling by the transforming growth factor-beta (TGF-beta) family of cytokines. We show that aortic aneurysm in a mouse model of MFS is associated with increased TGF-beta signaling and can be prevented by TGF-beta antagonists such as TGF-beta-neutralizing antibody or the angiotensin II type 1 receptor (AT1) blocker, losartan. AT1 antagonism also partially reversed noncardiovascular manifestations of MFS, including impaired alveolar septation. These data suggest that losartan, a drug already in clinical use for hypertension, merits investigation as a therapeutic strategy for patients with MFS and has the potential to prevent the major life-threatening manifestation of this disorder.
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PMID:Losartan, an AT1 antagonist, prevents aortic aneurysm in a mouse model of Marfan syndrome. 1660 Nov 63

Aortic dissection and aortic intramural hematoma are highly lethal diseases occurring mostly in patients with arterial hypertension or Marfan syndrome. Transesophageal echocardiography (TEE) is the diagnostic imaging procedure of choice in the unstable patient. Hallmarks of dissection are the presence of an intimal membrane, or flap, dividing a true and a false lumen, entry and reentry tears, periaortic hematoma, pericardial effusion or tamponade, and aortic regurgitation in dissection of the ascending aorta. TEE allows detection of involvement of the ascending aorta, which places the patient in an extremely high-risk group and necessitates urgent operation. The accuracy of TEE is similar to computed tomography and magnetic resonance imaging, with the added benefit of being feasible at the bedside or in the operating room. Precautions have to be taken during TEE to avoid an abrupt rise in blood pressure. Aortic intramural hematoma is often a precursor of classic dissection or accompanies it and can also be diagnosed by TEE, although the diagnosis is frequently difficult.
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PMID:Assessment of aortic dissection and hematoma. 1670 40

Medico-legal post-mortems referred to the Department of Pathology, for the histopathological examination, revealed six cases of acute aortic dissection--two in isolation, three in combination with congenital bicuspid aortic valve; and one isolated case of congenital bicuspid aortic valve. One case of isolated aortic dissection was associated with Marfan's syndrome; and one case of aortic dissection with bicuspid aortic valve was associated with polycystic kidneys. History of hypertension could be elicited in two cases. Cystic medial degeneration of aorta was seen in three cases; one of which was associated with Marfan's syndrome. All five cases of aortic dissection belonged to type II of DeBakey classification.
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PMID:Aortic dissection and bicuspid aortic valve: an autopsy study. 1700 77

We describe a 19-year-old male, with a family history of both systemic lupus erythematosus and Marfan syndrome, who had a history of bruising easily and skin lesions since childhood. He had a spontaneous colonic perforation at the age of 16 years, followed 3 years later by sudden development of bilateral renal infarctions and hypertension, which on angiography were found to be due to dissection of both renal arteries. Transient elevations of 3 types of antiphospholipid antibodies (aPL) were detected. Skin biopsy showed typical elastosis perforans serpiginosa. The history together with the generalized connective tissue phenotype, histology, and angiographic features combined to establish a diagnosis of vascular Ehlers-Danlos syndrome, type IV; the body habitus resembled the phenotypically-related condition of Marfan syndrome. The coincidental finding of transient aPL elevations combined to make this a difficult diagnostic and clinical management problem.
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PMID:Ehlers-Danlos syndrome type IV in a young man. 1701 25

Iliorenal bypass is a nonanatomic means of renal revascularization usually performed in high-risk patients. Its efficacy was assessed in this review of 35 patients (17 males and 18 females, two children and 33 adults) ranging in age 8-84 years, who were subjected to 41 iliorenal bypasses at the University of Michigan Hospital during 1975-2003. Renal artery lesions included arteriosclerosis (n = 20), developmental narrowing (n = 10), arterial fibrodysplasia (n = 3), penetrating trauma (n = 1), and aortorenal dissection associated with Marfan disease (n = 1). All patients had hypertension attributed to their renal artery disease. Twenty patients exhibited renal insufficiency (serum creatinine >1.8 mg/dL). Primary reasons for selecting an iliorenal reconstruction over a more conventional open revascularization included advanced aortic arteriosclerosis (n = 9); prior aortoaortic, aortoiliac, or aortofemoral reconstruction (n = 7); a small aortic aneurysm not justifying aortic surgery (n = 6); prior aortorenal surgery (n = 6); congenital abdominal aortic coarctation (n = 4); a hostile retroperitoneum (n = 2); or compromised cardiac status (n = 1). Eleven patients had prior ipsilateral renal artery interventions. Iliorenal bypasses were to the right kidney (n = 20), the left kidney (n = 9), and bilateral (n = 12). Conduits were saphenous veins (n = 29), synthetic prostheses (n = 11), or direct renal artery-iliac artery reimplantation (n = 1). Initial bypass patency was 93%. Follow-up averaged 7.5 years. Three early and six late graft complications resulted in eight secondary operations. The mean preoperative and postoperative serum creatinine of all 35 patients did not vary (1.9 vs. 1.8 mg/dL), although on an individual basis renal function improved in eight, remained stable in 21, and deteriorated in six patients. The series' mean preoperative blood pressure of 180/97 mm Hg decreased postoperatively to 140/78 mm Hg (P < 0.001). Hypertension was cured in three patients, improved in 27, and became worse in four. Antihypertensive medication numbers decreased postoperatively, from a median of three to two (P < 0.0001). Surgical mortality was limited to one patient succumbing from perioperative intestinal infarction. Iliorenal bypass is an effective means of renal revascularization in patients not amenable to more conventional open or transluminal procedures.
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PMID:Iliorenal bypass: indications and outcomes following 41 reconstructions. 1734 28

Aortic dissection occurs when blood penetrates the aortic intima and forms an expanding hematoma within the vessel wall, separating the intima and media to create a so-called false lumen. Aortic dissection has been estimated to cause one of every 10,000 hospital admissions. The survival rate of untreated patients with aortic dissections is dismal, with a 2-day mortality of up to 50%. The medical conditions predisposing a patient to aortic dissection are hypertension, advanced age, sex, Marfan syndrome and some forms of congenital heart disease. Pregnancy and delivery are not considered major risk factors for the disease. We report here two consecutive cases of type A aortic dissection (Stanford classification) in young puerperal patients after elective caesarean section.
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PMID:Two consecutive cases of type A aortic dissection after delivery. 1758 25

Angiotensin-converting enzyme inhibitors (ACE-I) and angiotensin receptor blockers (ARB) posses multiple beneficial effects such as cardioprotection, cerebroprotection, nephroprotection which provide opportunity to select the most suitable drug for the target vascular bed (e.g. coronary, or cerebral circulation). In some clinical settings, combined therapy ACE-I with ARB (double blockage of the renin-angiotensin-aldosteron system) may appear the most effective. These drugs (especially ARB) may successfully prevent atrial fibrillation and play a protective role in metabolic syndrome. Recently, it has been demonstrated that losartan is able to inhibit vasodilatation of the aorta in Marfan syndrome, which might prevent sudden death due to aorta rupture. An increasing role of ARB is most beneficial in hipotensive therapy (inhibition/regression of hypertension-related organ damage). With particular interest, results of the ONTARGET study are being awaited. This study is focused on the effect of double blockage (ramipril and telmisartan) on reduction of the occurrence of myocardial infarction, stroke, and heart failure.
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PMID:[New therapeutic targets for ACE inhibitors and angiotensin receptor blockers]. 1772 75

Medullary sponge kidney (MSK) is a congenital anomaly characterized by dilatation of the collecting ducts of Bellini associated with defective urinary acidification and concentration. Medullary nephrocalcinosis/ nephrolithiasis is the usual presentation in adults, however neonatal and childhood cases are being reported with increasing frequency. Among the conditions associated with MSK are Beckwith-Wiedemann syndrome/hemihyperplasia (13%), horseshoe kidney, congenital small kidney, hyperparathyroidism, Caroli syndrome, congenital hepatic fibrosis, Ehlers-Danlos syndrome, Marfan syndrome, immotile cilia syndrome, and arterial fibromuscular dysplasia. This article describes an adult female who underwent nephrectomy for renovascular hypertension due to multivessel fibromuscular dysplasia with small left kidney found to be associated with MSK.
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PMID:Medullary sponge kidney associated with multivessel fibromuscular dysplasia: report of a case with renovascular hypertension. 1820 94


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