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

Twenty percutaneous transluminal renal angioplasties were performed on 16 children (mean age 8.7 years) with hypertension secondary to renal artery stenosis (RAS). The aetiologies were neurofibromatosis (n = 1), Williams syndrome (n = 2), Takayasu arteritis (n = 1) and fibromuscular dysplasia (n = 12). The stenosis was isolated proximal or distal in 13 cases and multiple in 3 cases. Angioplasty resulted in a complete cure without medical treatment in 9 cases. Angioplasty allowed a reduction of medical treatment in two single stenoses, but was ineffective in all cases of multiple stenoses. Our findings show that angioplasty of RAS in children is an effective treatment when the stenosis is isolated, with a 69 % success rate. It seems ineffective in case of multiple stenoses (three cases).
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PMID:Percutaneous transluminal angioplasty of renal artery stenosis in children. 942 77

From 1971 through 1996, 16 patients were operated on for atypical aortic coarctation. Three of them (two women, 18 and 27 years old, one boy, 13 years old) had typical signs of neurofibromatosis type I (NF1). All had renovascular hypertension due to suprarenal and/or interrenal aortic stenosis. Two had additional bilateral renal artery stenoses, one with a poststenotic aneurysm. In all patients a thoraco-abdominal aorto-aortic bypass was implanted. The renal artery stenoses were bridged by two aorto-renal, a prothesio-renal, and a spleno-renal bypass. Only one kidney had to be explanted 13 years after revascularization for a recurring hilar aneurysm after ex-situ reconstruction was found to be impossible. At their last follow-up examination (at 16, 14, and 9 years) all patients were normotensive without medication and had normal serum-creatinine measurements. Angiography or spiral-CT done in all patients showed normal function of all bypasses. In literature we found another 27 patients with neurofibromatosis operated on for atypical aortic coarctation. In these young and otherwise healthy patients, even complex reconstructions can be performed with a low rate of complications and excellent long-term results. Surgical therapy of all relevant stenoses (at best in a single procedure) is necessary for complete relief of renovascular hypertension. Consequent follow-up is advised for the detection of possible late complications, especially after exclusion of aneurysms.
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PMID:[Atypical aortic coarctations in type I neurofibromatosis]. 945 80

A 31-y-old black man with neurofibromatosis, alcoholism and hypertension was admitted because of abdominal pain, hematemesis and cough. In the hospital he had prolonged fever and developed a multiorgan crisis. Despite thorough investigation, no infectious cause for fever was found. Urinary catecholamines and metabolites were markedly elevated. Computerized tomography revealed a mass abutting the left kidney. A diagnosis of pheochromocytoma was made, and as soon as treatment with phenoxybenzamine and propranolol was begun, the fever resolved. Serum interleukin-6 (IL-6) concentration was initially elevated, decreased after the start of adrenergic blockade, and gradually fell to an undetectable level after surgery. These observations suggest that interleukin-6 might have been causally related to the patient's fever and possibly the multiorgan crisis.
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PMID:Interleukin-6 in the fever and multiorgan crisis of pheochromocytoma. 957 53

Since fiscal year 1991, the U.S. Human Genome Project has spent $170.6 million in federal funds to help isolate genes associated with Huntington's disease, amyotrophic lateral sclerosis, neurofibromatosis types 1 and 2, myotonic dystrophy, and fragile X syndrome and to localize genes that predispose people to breast cancer, colon cancer, hypertension, diabetes, and Alzheimer's disease. Now comes the hard part. Biology's 21st century megaproject starts to look relatively manageable compared to another challenge facing the enterprise: sorting out ethical, legal, and social issues associated with using this information. "The Human Genome Project," wrote Senior Editor Barbara Jasny in the October 1 Science editorial, stretches "the limits of the technology and the limits of our ability to ethically and rationally apply genetic information to our lives."
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PMID:Ethical, legal, and social issues of the Human Genome Project: what to do with what we know. 971 68

A 26-year-old man who had neurofibromatosis type-2 with symptoms of unexplained optic disc edema is reported. Magnetic resonance imaging (MRI) revealed bilateral acoustic schwannomas. Obstructive hydrocephalus, however, was not evident in spite of his severe disc edema and visual loss. After partial removal of the right acoustic schwannoma, symptoms of intracranial hypertension, such as vomiting and headache, developed and MRI demonstrated evidence of obstructive hydrocephalus. Placement of a ventricular-peritoneal shunt relieved the symptoms of intracranial hypertension, but visual acuity in his left eye was reduced to hand motion due to secondary optic atrophy. In patients with similar symptoms it is suggested that, in addition to tumor removal, early treatment to decrease intracranial pressure should be considered when visual function is progressively impaired by the symptoms of prolonged papilledema.
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PMID:Severe optic disc edema without hydrocephalus in neurofibromatosis 2. 982 67

Neurofibromatosis Type 1 produces a broad spectrum of clinical manifestations as a result of widespread dysplasia of mesodermal and neuroectodermal tissues. One of the most serious aspects of the disease relates to the arterial involvement that may occur. We report a case of severe stenosis of the internal carotid artery and intracerebral hemorrhage associated with neurofibromatosis Type 1. A 49-year-old female was admitted to our hospital after she had suddenly become comatose. On admission, she demonstrated a decerebrate posture in response to painful stimuli, and was assessed as grade 200 according to the Japan Coma Scale. Physical examination disclosed widespread cutaneous neurofibromas and cafe-au-lait spots. Computed tomography of the head revealed a right putaminal extensive hematoma, with a maximum diameter of 7 cm. The hematoma was removed. After this surgical treatment, cerebral angiography was performed. It showed severe stenosis of the terminal portion of the right internal carotid artery associated with a fine telangiectatic network, indicating the presence of moyamoya vessels in the basal ganglia. Although intracranial hemorrhage associated with neurofibromatosis type 1 is a rare condition, fine telangiectatic collateral vessels caused by occlusive cerebrovascular disease, intracranial aneurysms, brain tumors, or hypertension caused by pheochromocytoma or stenosis of the renal artery should be considered as the cause of hemorrhage.
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PMID:[Severe stenosis of the internal carotid artery and intracerebral hematoma associated with neurofibromatosis type 1: a case report]. 1002 86

Arterial hypertension in patients with neurofibromatosis is most often due to an associated pheochromocytoma. In rare cases the etiology of arterial hypertension is renovascular. Surgical treatment is mandatory when the stenosis is located in the proximal segment of the vessel. Angioplasty is hazardous in this setting due to the fibrotic nature of the culprit lesion. The authors report a case of ostial narrowing of the right renal artery in a 16-year-old girl with severe arterial hypertension and neurofibromatosis. The operation consisted of resection of the culprit lesion and reimplantation of the renal artery on the aorta. Postoperatively her blood pressure returned to normal.
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PMID:[Renovascular arterial hypertension in the framework of von Recklinghausen's neurofibromatosis. Apropos of a case]. 1009 36

Pheochromocytoma is observed with higher frequency in patients with von Recklinghausen neurofibromatosis. We report a 36 years old female with von Recklinghausen neurofibromatosis who developed mild hypertension during the fourth month of pregnancy. A cesarean section was performed at 37 weeks of pregnancy. Thereafter, the patient presented severe hypertensive and hypotensive crises, sinus tachycardia and fever. No evidences of an infectious disease were found. Abdominal ultrasound examination showed a right adrenal mass of 7 x 5 cm. High levels of urinary cathecolamines confirmed the diagnosis of pheochromocytoma. After three weeks of prazosin therapy, the patient was operated. During the surgical procedure, an encapsulated pheochromocytoma was found and excised. A right renal atrophy and renal artery thrombosis were also found and a nephrectomy was done. Postoperative evolution was uneventful and the patient remains with normal blood pressure levels six months after the operation.
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PMID:[Pheochromocytoma and von Recklinghausen neurofibromatosis: postpartum crisis and renal artery thrombosis]. 1034 81

Percutaneous transluminal renal angioplasty (PTRA) alone or in combination with stent implantation, is increasingly used as an alternative technique to surgical revascularization for treatment of renal artery stenosis (RAS) wich may cause hypertension or jeopardize renal function. Herein we report the results obtained with 305 PTRAs performed in 242 hypertensive patients, 144 of whom had atherosclerotic RAS, 69 fibromuscolar dysplasia, 15 Ras in transplanted kidneys, 6 restenosis in surgically revascularized kidneys, 4 Takayasu arteritis and 4 neurofibromatosis. Stents were implanted in 68 cases, mostly in atherosclerotic stenoses. The technical success was achieved in 261 arteries (85.6%), with 33 failures (10.8%) and 11 (3.6%) procedures not completed for anatomical reasons. PTRA related complications were observed in 23 cases (7.5%), but no fatalities occurred. An overall benefit on blood pressure control was observed in 41% of patients with atherosclerotic RAS and in 68% of those with fibromuscolar dysplasia. It appears that independently from the ethiology PTRA is technically effective in correcting RAS; yet the position of PTRA with respect to that of medical or surgical treatment needs to be better delineated through randomized, controlled studies aimed at comparing the clinical efficacies of these different approaches.
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PMID:Interventional radiology in the treatment of renal artery stenosis. 1041 43

Type 1 neurofibromatosis (NF1) is the most frequently observed phacomatosis, but involvement of arterial trunks is uncommon. Expression depends on the localization and is not easily related to the causal condition. Seven patients with type 1 neurofibromatosis developed vascular manifestations (table I) disclosed by hypertension (n = 2) digestive angina (n = 1), arterial rupture (n = 1) and aneurysm of the subrenal aorta (n = 1). The diagnosis of NF1 was clear in 5 cases; in 2 cases, the diagnosis could only be established on the basis of pathology findings demonstrating dysplasia of the media with voluminous periadventitial hypertrophic nerves (table II). All the large arteries can be involved in NF1. A complete vascular work-up is needed to identify multiple arterial localizations as found in two of our cases. Thoraco-abdominal stenosis was observed in 5 cases leading, in 2 cases, to coarctation with a hemodynamic and functional impact requiring aortic revascularization. The most frequently observed localization involves the renal arteries: 3 of our patient had occlusive lesions of the renal arteries and in 2, aneurysms were observed. Three of our patients (including 2 of the preceding), had major occlusion of digestive arteries. Three other cases revealed an aneurysm of inflammatory subrenal aorta, a rupture of the iliac into the inferior vena cava and a rupture covered by a subclavian aneurysm. The indication for surgery depends on the arterial signs of associated complications (5 of our cases). In one case surgery was indicated to prevent rupture of a splenic artery aneurysm and an aneurysm of the subrenal abdominal aorta. Two cases were treated by exclusion (ilio-cava fistula) or excision (splenic aneurysm); renal or digestive revascularization was performed with arterial or venous autografts in young patients (3 cases). One extensive abdominal coarctation was repaired with a PTFE graft as were the subclavian and subrenal aorta aneurysms. One patient with an ilio-cava fistula died from collapsus. Long-term results of the revascularizations are satisfactory with good control of the hypertension and total regression of the digestive angina. Fibrodysplasia of the renal or digestive media occurring alone or thoraco-abdominal coarctation should suggest NF1 and lead to a complete work-up to identify other arterial localizations. Patients should be followed regularly to prevent complications which in case of rupture can be life-threatening.
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PMID:[Arterial complications of neurofibromatosis]. 1058 77


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