1/45. A case of neurofibromatosis type 1 with an aldosterone-producing adenoma of the adrenal.Neurofibromatosis type 1 is a phacomatosis. Neurofibromas are the most common tumours associated with the disease, and along with other tumours, make neurofibromatosis type 1 the most common tumour predisposing syndrome in humans. hypertension may be coincidental, but at least two specific neurofibromatosis related causes must be considered, namely neurofibromatous involvement of the renal artery and pheochromocytoma. We have described the first known case of a patient with neurofibromatosis type 1 who developed hypertension due to an aldosterone-producing adenoma of the adrenal. The question of whether this association was coincidental or due to the tumour predisposition of neurofibromatosis type 1 was debated.- - - - - - - - - - ranking = 1keywords = hypertension (Clic here for more details about this article) |
2/45. Neurofibromatosis and hypertension. A case report.The case of a 12-year-old boy with neurofibromatosis, kyphoscoliosis, and hypertension is reported. The association of neuroflibromatosis and kryphoscoliosis is well known. There are no reports on the association of neurofibromatosis and hypertension in the orthopaedic literature. The cause was found to be intrarenal vascular abnormalities which are characteristic of neurofibromatosis. Other possible causes of hypertension in this disease are discussed. This patient was successfully treated by a partial nephrectomy.- - - - - - - - - - ranking = 7keywords = hypertension (Clic here for more details about this article) |
3/45. von Recklinghausen disease complicated by pulmonary hypertension.Two patients with von Recklinghausen disease (neurofibromatosis type 1) were admitted to the hospital because of progressive heart failure. Both patients had prominent pulmonary hypertension revealed on cardiac catheterization. A lung perfusion scan did not show any gross defect. There were no underlying causes of pulmonary hypertension in either patient, such as chronic lung disease, congenital or acquired heart disease, deep vein thrombosis, or systemic hypercoagulable states. There may be an unrecognized association between von Recklinghausen disease and pulmonary hypertension.- - - - - - - - - - ranking = 7keywords = hypertension (Clic here for more details about this article) |
4/45. Middle aortic syndrome: distal thoracic and abdominal coarctation, a disorder with multiple etiologies.BACKGROUND: Congenital coarctation of the thoracic aorta at the ligamentum arteriosum or the aortic arch is well recognized. But a much less common variety (0.5% to 2.0%) of aortic coarctation is located in the distal thoracic aorta, or abdominal aorta, or both and is often called "middle aortic syndrome" or "mid-aortic dysplastic syndrome." These types of aortic coarctation are most often secondary to a form of granulomatis vasculitis commonly known as Takayasu's disease in this country or aortitis syndrome in japan. No single genesis explains every case and beside vasculitis as a cause, some are thought to be congenital in origin and others are associated with von Recklinghausen's disease. STUDY DESIGN: Eight patients with severe hypertension or claudication secondary to middle aortic coarctation were studied with aortograms and subsequently treated by vascular reconstruction procedures. RESULTS: Vascular reconstructions consisted of aortoaortic bypass, aortic resection with interposed grafting, reanastomotic resection of renal arteries into prosthetic grafts, and renal artery bypass with autogenous material. All eight patients' grafts have remained patent, with followups of 4 to 9 years, with relief of hypertension and claudication. Although Takayasu's disease can be progressive, aggressive surgical treatment in eight patients followed for 4 to 8 years postoperatively demonstrates that severe hypertension, claudication, or both are important indications for revascularization. CONCLUSIONS: Whatever the cause, assuming that active aortic inflammation has been medically treated and is in a burned-out state, patients with abdominal coarctation who have symptomatic renovascular hypertension, claudication, or both are good candidates for revascularization. Although surgical repair is more difficult than with congenital thoracic coarctation, because aortic walls are fibrotic and often also involve the renals, all eight of our patients had successful longterm correction of their hypertension and coarctation.- - - - - - - - - - ranking = 12.521584772271keywords = renovascular, hypertension (Clic here for more details about this article) |
5/45. eclampsia complicating a pregnancy with neurofibromatosis. A case report.Neurofibromatosis occurs in approximately 1 in 3,000 births and is known to be associated with the development and/or worsening of hypertension in pregnancy. A woman was treated for eclampsia with neurofibromatosis and had a complicated course.- - - - - - - - - - ranking = 1keywords = hypertension (Clic here for more details about this article) |
6/45. A case of neurofibromatosis associated with clitoral enlargement and hypertension.We report a case of clitoral and renovascular involvement of neurofibromatosis resulting in an enlarged phallus with juvenile hypertension. The patient was successfully treated by removal of the clitoral tumor and nephrectomy. This is the first of 15 reported cases with clitoral involvement, that showed concurrent renovascular hypertension.- - - - - - - - - - ranking = 21.043169544542keywords = renovascular, hypertension (Clic here for more details about this article) |
7/45. renal artery stenosis and aneurysms associated with neurofibromatosis.renal artery lesions associated with neurofibromatosis may involve stenosis and aneurysm formation at all levels of the renal artery to the intraparenchymal branches, and usually are associated with hypertension. A 13-year-old boy with type I neurofibromatosis and severe hypertension presented with multiple aneurysms and multiple stenotic lesions in the renal artery and segmental arteries. The patient underwent ex-vivo renal artery repair with autologous hypogastric artery and autotransplantation to the iliac fossa and was clinically improved. The characteristic histologic findings are presented. A review of the recent literature comparing different treatment modalities for renovascular hypertension in children with neurofibromatosis suggests that surgery remains the best treatment alternative.- - - - - - - - - - ranking = 10.521584772271keywords = renovascular, hypertension (Clic here for more details about this article) |
8/45. pheochromocytoma associated with adrenocortical tumor in the same gland. Two case reports and literature review.Pheochromocytomas are catecholamine-producing neuroendocrine tumours arising from chromaffin cells of the adrenal medulla or extra-adrenal paraganglionic system that show 2 distinctive features, rarity and clinical variability. pheochromocytoma occasionally is associated with pathological lesions of the adrenal cortex. We present 2 cases of patients referred to our hospital with a finding of clinical suspected pheochromocytoma. Both of them were hypertensive; the first patient with typical symptoms of pheochromocytoma and the second patient with chest pain and hypertension resistant to pharmacological treatment. The diagnosis of pheochromocytoma was confirmed in both cases with laboratory analysis and the lesion was achieved by employing 3 imaging techniques: computed tomography (CT), magnetic resonance imaging (MRI) and scintigraphy with (123)I-metaiodobenzilguanidine (MIBG). The patients underwent adrenalectomy and in the same adrenal gland we found a pheochromocytoma associated with a nonfunctioning cortical adenoma. As far as we know few cases with this association are available in the literature.- - - - - - - - - - ranking = 1keywords = hypertension (Clic here for more details about this article) |
9/45. Acute renal insufficiency due to renal infarctions in a patient with neurofibromatosis.Manifestations of neurofibromatosis in the skin, the eye, and the skeletal and nervous systems have been well documented since the disease was first described in 1882. Stenosing vascular lesions as complications of neurofibromatosis were first reported in 1945. They are being increasingly recognized and most commonly involve the renal artery. renal artery stenosis (usually proximal), intraparenchymal renal arterial abnormalities, and coarctation of the abdominal aorta often lead to hypertension. However, despite reports of bilateral and severe renal artery disease, renal infarction and resulting renal insufficiency have not been described. We present the case of a 35-year-old woman with neurofibromatosis and chronic hypertension associated with narrowing of right intrarenal arteries. The patient had two separate episodes of left renal infarction documented clinically and radiographically. The second infarct resulted in renal insufficieny. There was no hypercoagulopathy or source for embolism. This case suggests that renal infarction and renal insufficiency are additional complications of neurofibromatosis.- - - - - - - - - - ranking = 2keywords = hypertension (Clic here for more details about this article) |
10/45. Neurofibromatosis associated with renovascular hypertension due to stenosis and aneurysm of the left renal segmental artery: report of a case.We report a case of renovascular hypertension due to renal segmental artery stenosis and aneurysm seen in a 16-year-old boy with neurofibromatosis. hypertension was successfully treated with partial nephrectomy. Histopathologic examination disclosed proliferation of arterial intima not only in the stenotic portion and the aneurysm but also in intralobular arteries of the kidney. review of the literature revealed that treatment for such patients has varied.- - - - - - - - - - ranking = 42.607923861356keywords = renovascular, hypertension (Clic here for more details about this article) |
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