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

Three patients, ages 13 months, 13 years, and 16 years, with renovascular hypertension complicating neurofibromatosis underwent vascular reconstruction with good results. A review of these patients, plus 47 additional patients from the literature, constitutes the basis of this report. The standard intravenous pyelogram revealed abnormalities in only 46 per cent of the patients. Definitive diagnosis required angiography and selective renal vein renin determinations. Unilateral and bilateral lesions were seen with equal frequency. Associated arterial abnormalities were common. Medical treatment and balloon angioplasty failed to control the blood pressure in all patients. Surgical therapy, either nephrectomy or vascular reconstruction, cured or significantly improved 95 per cent of the patients. Although one primary goal of operation is renal salvage, 40 per cent of the patients in this review underwent nephrectomy to control their hypertension.
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PMID:Renovascular hypertension complicating neurofibromatosis. 391 94

A 14-year-old boy presented with hypertension and symptomatic cervical root neurofibromatosis. Isolated thoracic aortic coarctation and large collaterals adjacent to the cervical spine were identified. Surgical correction of the coarctation allowed safe excision of the neurofibroma and reversal of hypertension. Hypertension in the presence of neurofibromatosis requires evaluation of a number of correctable etiologies.
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PMID:Hypertension from isolated thoracic aortic coarctation associated with neurofibromatosis. 392 8

A 34-year-old white man with generalized neurofibromatosis was found to have severe renal vascular hypertension due to a coarctation of the abdominal aorta and bilateral renal artery stenosis with saccular aneurysms. Increased renal venous renin activity showed the active involvement of the renin-angiotensin system in maintaining the hypertension. Because the patient refused surgical treatment, antihypertensive treatment with Captopril, a specific inhibitor of the angiotensin converting enzyme was used, resulting in normal blood pressure being restored over an 18 month observation period.
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PMID:Secondary hypertension and neurofibromatosis: bilateral renal artery stenosis and coarctation of the abdominal aorta. 392 61

Between 1970 and 1982, 50 patients (38 male and 12 female) underwent revascularization of 51 occluded renal arteries. Ages ranged from 8 to 71 years (mean 54.6 years). Occlusion was caused by atherosclerosis in 43 patients, fibromuscular dysplasia in three, chronic dissection in two, abdominal aortic coarctation in one, and neurofibromatosis in one. Contralateral renal artery occlusive disease occurred in 22 patients. Extrarenal atherosclerosis occurred in 44 patients. Mean preoperative serum creatinine level ranged from 0.5 to 8.4 mg/dl (mean 1.9 mg/dl). No patient required preoperative dialysis. Length of the involved kidney ranged from 8.4 to 14.5 cm (mean 11.5 cm). Indication for renal revascularization was hypertension in 49 patients and preservation of renal function in one. Renal artery bypass was performed in 36 patients, renal artery endarterectomy in six, transaortic endarterectomy in five, and reimplantation of the renal artery in three. Simultaneous revascularization of the contralateral renal artery was performed in 20 patients. There were three operative deaths. At hospital dismissal, hypertension had improved in 45 of 46 patients. Follow-up periods ranged from 4 months to 12 years (mean 50.2 months). Thirty-four patients remained normotensive, five still had less hypertension, and seven became worse. These data demonstrate that revascularization of an occluded renal artery can be effective in controlling hypertension and that this effect is durable in the majority of patients.
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PMID:The occluded renal artery: durability of revascularization. 396 46

Angioplasty was carried out in four children with hypertension and renal artery stenosis, including one patient with neurofibromatosis and one patient with multiple arterial stenoses. Two patients did not respond. In the patient with neurofibromatosis the stenosis persisted unchanged despite multiple balloon inflations. In one patient hypertension persisted despite successful dilatation, and was probably related to longstanding contralateral kidney disease. Two patients had excellent blood pressure response to dilatation. One of these patients required redilatation of bilateral recurrent stenoses.
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PMID:Percutaneous transluminal angioplasty for treatment of renal artery stenosis in children. 624 Mar 98

A 14-year-old girl with neurofibromatosis presented with severe hypertension. She was subsequently found to have a cerebellar glioblastoma multiforme and vascular lesions producing coarctation of the abdominal aorta and 50% and 95% stenosis of the left and right renal arteries respectively. No evidence of pheochromocytoma was found. After removal of the cerebellar tumor, marked amelioration of the hypertension suggested that the tumor had a major role in the pronounced elevation of her blood pressure. Patients who have both neurofibromatosis and hypertension should be carefully evaluated for these several potential lesions.
Hypertension
PMID:Hypertension and neurofibromatosis. Case report. 629 87

Four pregnant patients with neurofibromatosis (Von Recklinghausen's disease) either developed hypertension during pregnancy or had an exacerbation of a pre-existing chronic hypertension. Two patients required early termination of pregnancy for severe hypertension; a third had severe intrauterine growth retardation resulting in intrauterine fetal death; and one had an otherwise uneventful pregnancy and a live healthy infant. Because of the association between neurofibromatosis and hypertension, patients with neurofibromatosis require special antenatal care and management.
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PMID:Neurofibromatosis and severe hypertension in pregnancy. 640 15

Vascular lesions in addition to pheochromocytoma are now recognized as causes of hypertension associated with neurofibromatosis. We have analyzed hospital charts of 106 pediatric patients with neurofibromatosis, eight of whom were noted to have been hypertensive on at least one occasion. Of these eight patients, five had vascular lesions involving the renal vasculature, one had obstructive uropathy and two had transient hypertension associated with traction and pain. Approximately 5% of pediatric patients with neurofibromatosis have vascular lesions causing hypertension.
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PMID:Renovascular hypertension in pediatric patients with neurofibromatosis. 641 42

Neurofibromatosis in children occurs uncommonly and involvement of the bladder is exceedingly rare. The syndrome is transmitted as an autosomal dominant trait and is characterized by cutaneous pigmentation and tumors of neural crest origin. Hypertension has been documented in association with neurofibromatosis in the form of renovascular disease in children and with pheochromocytomas in adults. We report on 2 children with extensive neurofibromas of the bladder, upper tract damage and genital enlargement. One case was misdiagnosed as posterior urethral valves and the other case as clitoral hypertrophy. The literature of urogenital neurofibromatosis is reviewed with particular attention to bladder lesions, hypertension, malignant potential and treatment options.
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PMID:Genitourinary neurofibromatosis in childhood. 641 45

Two cases of spontaneous hemothorax in pregnant women with neurofibromatosis are reported. Effects of pregnancy on the clinical course of neurofibromatosis are known to include worsening of cutaneous lesions, increased incidence of hypertension, and renal artery rupture. However, vessel wall rupture into the pleural cavity in areas of vascular neurofibromatosis is previously unreported. Contributing factors may include normal hemodynamic changes of the puerperium and pathological alterations of preexisting areas of neurofibromatosis. Because the incidence of vascular neurofibromatosis is not known and is difficult to diagnose, the serious morbidity and mortality encountered in this group place all pregnant patients with neurofibromatosis in a high-risk obstetric category.
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PMID:Neurofibromatosis and spontaneous hemothorax in pregnancy: two case reports. 642 69


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