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

This paper reports the case of a girl who, at the age of 7 years, presented with a saccular aneurysm of a brachial artery. Over the following 8 years a further 15 aneurysm appeared, involving limb and visceral arteries and the aorta. Some, but not all, of these aneurysms have been operated upon. Complications included renovascular hypertension due to bilateral intrarenal aneurysms. She had been followed up for 15 years. Three other similar cases have been found in the medical literature, and these are also referred to. In two of these cases the affected persons also exhibited some of the other connective tissue anomalies associated with the Ehlers--Danlos syndrome. These were not present in our patient. The relationship between the syndrome of multiple congenital aneurysms and the Ehlers--Danlos syndrome is discussed.
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PMID:Multiple congenital aneurysms in childhood: report of a case. 66 48

One hundred and twenty patients with arterial hypertension and 3 cases of fibromuscular displasia of the renal arteries (FDRA) (group D) were studied; besides the usual arterial hypertension work up, a search of Ehlers-Danlos syndrome stigmata was carried out in all patients. The population of 120 hypertensive patients were classified in 3 groups: group A, fromed by 104 subjects without EDS stigmata; group B, constitued by 7 cases with hypermobility of one to three metar-carpophalangic joints and group C in which 9 subjects with hipermobility of more than 3 metacarpophalangic joints were included. Neither case of groups A and B showed arteriographic signs of FDRA. In 4 cases of group C there were arteriographic evidence of FDRA (3.3% of the whole population) and stigmata of SED (2 cases with skin biopsy positive for this entity). Two cases of group D showed stigmata of SED and a positive skin biopsy; the other case had a normal histology of skin and no clinical signs of SED. The presence of SED stigmata in a patient with arterial hypertension should alert the clinician in the diagnosis of FRDA; in such a case it is recommended that a renal arteriography be performed. In this paper similarities of SED of the arterial type and some cases of FDRA are remarked and it is proposed the both entities share the same or a very closed molecular collagen defect.
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PMID:[Further cases of Ehlers-Danlos syndrome of the renovascular dysplasic variety]. 93 46

This is the case of a 34-year-old woman with Ehlers-Danlos syndrome whose cardiopulmonary manifestations are the following: Prolapse of mitral and tricuspid valves. Aneurysmal dilatation of main arteries without aortic or pulmonary insufficiency. Disturbances in pulmonary function tests and pulmonary arterial hypertension. The diagnosis was verified by skin biopsy and an electron microscopic study. Due to the clinical and histopathological characteristics, we have considered this case to be a non-specified type of the 10 varieties described up to now, and have decided to report it also because of the interesting findings in the hemodynamic and pulmonary function tests.
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PMID:[Cardiovascular abnormalities in Ehlers-Danlos syndrome. Report of a case]. 278 94

The authors describe the case of a Marfan syndrome who presented a giant intracranial internal carotid aneurysm associated with elongation and tortuosity of internal carotid and vertebral arteries on both sides. The skin microscopic examination showed fragmentation and distorsion of elastic fibers. There was no microscopic study of the vessels. Extra and intracranial vascular abnormalities are rarely reported in marfan syndrome: cardiovascular changes are seen mostly in aorta and pulmonary artery. In the literature are reported some giant aneurysms, dissections and dilatations of carotid, basilar and vertebral arteries. Usually the microscopic examination of the vessels show cystic medial necrosis. Other connective tissue diseases (pseudo-xanthoma elasticum, Ehlers-Danlos syndrome, progeria) are described with such clinical abnormalities. Reference is made to the possibility of unknown histological and chemical lesions weakening the vessels in patients without connective tissue disorders and presenting with arterial dissection or arterial aneurysm. So vessels would be more sensible to aging, arteriosclerosis or hypertension.
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PMID:[Aneurysm of the internal carotid artery and cervical mega-dolicho-arteries in Marfan syndrome]. 323 Dec 95

The interaction of elevated blood pressure and aortic metabolism in the genesis of aortic dissection is uncharacterized. A kindred with fatal familial aortic dissection in association with precocious systemic hypertension and in absence of a definable connective tissue syndrome has undergone genealogical, clinical, pathological, and biochemical evaluation. Six family members spanning three generations have died of acute dissection. Five men died at a mean age of 28 years (range 22-34), while the proband's paternal grandmother died at 62 years of age. All were hypertensive. A constellation of subtle clinical features points toward deficient integrity of connective tissues; however, major hallmarks of known connective tissue syndromes including aortic root ectasia or aneurysms are absent. Studies of cultured dermal and aortae fibroblasts of two of the proband's brothers mitigate against Ehlers-Danlos IV syndrome. This family's susceptibility to aortic dissection reflects the synergistic liability of coexistent elevated blood pressure and metabolic abnormalities in the genesis of aortic degeneration.
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PMID:Familial aortic dissection in absence of ascending aortic aneurysms: a lethal syndrome associated with precocious systemic hypertension. 345 36

The association of the Ehlers-Danlos syndrome with many cardiovascular abnormalities is well known. To our knowledge, however, renovascular hypertension due to renal arterial aneurysms has not previously been reported in patients with this disease. We describe a patient with type IV Ehlers-Danlos syndrome who had multiple systemic and bilateral renal arterial aneurysms and hypertension. The hypertension could have been related to compression of renal tissue or arteries (or both) by the large aneurysms, associated stenoses, or small peripheral renal infarctions. A review of all patients in whom the Ehlers-Danlos syndrome had been diagnosed at our institution between 1967 and 1985 revealed that 4 of the 200 patients had hypertension. Three of these patients, and probably all four, had secondary causes of hypertension: Cushing's syndrome, renal insufficiency, or renovascular hypertension (in the present case). We conclude that hypertension is rare in patients with the Ehlers-Danlos syndrome and that, if present, it most likely is secondary hypertension. A thorough search for secondary causes of hypertension should be undertaken in these patients.
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PMID:Renovascular hypertension: a rare cardiovascular manifestation of the Ehlers-Danlos syndrome. 382 Nov 83

The Ehlers-Danlos syndrome (EDS) is a disorder of the connective tissue characterized by hyperextensible skin, loose jointedness, fragile tissues, bruising and bleeding diathesis. At least eight subtypes of EDS are recognized, each one with different clinical manifestations. On rare occasions EDS is associated with pregnancy. These patients are at risk for bleeding disorders and vascular, surgical and anesthetic complications as well as for premature labor, postpartum hemorrhage, bladder and uterine prolapse, abdominal hernias and wound dehiscence. We treated a pregnant patient for type 1 EDS and pregnancy-induced hypertension.
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PMID:Complications of the Ehlers-Danlos syndrome in pregnancy. A case report. 651 86

The association of Ehlers-Danlos type IV syndrome and acrogeria with renovascular hypertension is reported. Ultrastructural abnormalities observed in our case were different of those associated with acrogeria. We propose that acrogeria associated with Ehlers-Danlos type IV syndrome be a different disease of acrogeria of Gottron. The occurrence of renovascular hypertension in Ehlers-Danlos is unusual.
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PMID:[Ehlers-Danlos type IV syndrome presenting as renovascular hypertension]. 785 79

Arterial involvement is an important feature of the diagnosis and, above all, prognosis of heritable disorders of connective tissue. In pseudoxanthoma elasticum, a progressive occlusive syndrome is associated with hemorrhage and especially with gastrointestinal bleeding. Aneurysms are uncommon. Hypertension occurs frequently. Cutaneous signs (yellowish pseudo xanthomatous papules of the large folds) the ocular changes (angioid streaks) and pathology showing numerous, thickened, fragmented, disorganized, calcified elastic fibers in the deep dermis and arterial walls, allow the diagnosis to be made. In the heterogeneous group of Ehlers-Danlos syndromes, type IV is characterized by sudden spontaneous rupture of the large arteries. Aneurysms and carotido-cavernous fistulae are rather frequent. Owing to friability of the arterial walls, arteriograms and other procedure requiring arterial puncture may prove hazardous and surgery difficult. Such patients have an acrogeric morphotype, and thin, fragile skin, but cutaneous hyperelasticity and joint hyperlaxity are usually minimal. Pathology evidences collagen hypoplasia in the skin and arterial walls. The severity of Marfan syndrome is due to aortic involvement. A fusiform aneurysm of the ascending aorta represents a vital risk of rupture. Aortic root dilatation is associated and responsible of severe aortic regurgitation. Aortic dissection is also a serious threat. Improved surgical techniques for repairing a dilated or dissected aortic root with simultaneous replacement of the aortic valve increases the life expectancy of such patients. Dolichomorphism is the characteristic skeletal abnormality, particularly with arachnodactyly and upward ectopia lentis, which is almost bilateral, is a very frequent feature of Marfan syndrome. The most typical histological finding is aortic cystic median necrosis. The basic defect in Marfan syndrome concerns the fibrillin, whose gene is located on chromosome 15. The three diseases detailed in this paper constitute the main areas of this subject, but arterial involvement may occur in other inheritable disorders of connective tissue (osteogenesis imperfecta, cutis laxa, Werner syndrome, Menkes syndrome, etc).
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PMID:[Arterial involvements in hereditary dysplasia of the connective tissue]. 805 35

A 56-year-old female had pure regurgitation in all cardiac valves. Color Doppler echocardiography showed a regurgitant jet in all cardiac valves. The severity of regurgitation due to the prolapse in all valves was moderate. The patient had no history of rheumatic fever, ischemic heart disease, endocarditis or hypertension. Physical characteristics of the patient were neither of Marfan's nor Ehlers-Danlos' syndrome. The etiology of regurgitation in all cardiac valves of this patient may be due to multiple valve prolapse.
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PMID:An adult case with multiple cardiac valve prolapse and regurgitation. 832 22


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