1/9. Pulmonary hypertension secondary to neurofibromatosis: intimal fibrosis versus thromboembolism.Neurofibromatosis has been known to involve blood vessels throughout the body. Pulmonary involvement with interstitial fibrosing alveolitis has been described but no case of pulmonary vascular involvement has been reported to date. A 51 year old patient with cutaneous neurofibromatosis is described who presented with severe pulmonary hypertension and radiographic, scintigraphic, and angiographic evidence of chronic thromboembolic pulmonary hypertension. Severe intimal fibrosis consistent with vascular involvement with neurofibromatosis was found on endarterectomy with no evidence of pulmonary thromboembolism. Neurofibromatosis of pulmonary arteries should be considered as a possible cause of pulmonary hypertension.- - - - - - - - - - ranking = 1keywords = hypertension (Clic here for more details about this article) |
2/9. Renovascular hypertension associated with neurofibromatosis: two cases and review of the literature.The authors report two cases of renovascular hypertension associated with neurofibromatosis. A 19-year-old woman was admitted to our hospital with a complaint of abdominal pain and blood pressure of 180/120 mmHg. Examination revealed cafe-au-lait spots over her chest and extremities. Peripheral plasma renin activity (PRA) under basal conditions was 2.8 ng/ml/h and increased to 12.6 ng/ml/h after administration of 50 mg captopril. plasma and urinary catecholamines were normal. Selective renal angiography showed left aneurysmal dilatation of the segmentary branch and right renal artery stenosis with multiple aneurysmal affecting different branches. blood pressure was controlled by multiple drugs, including beta-blockers and angiotensin-converting enzyme inhibitor. Another patient, a 20-year-old woman, was admitted because of severe arterial hypertension, numerous cafe-au-lait spots, scoliosis, and mass over the right arm. PRA from the right renal vein was extremely elevated, and selective angiography demonstrated bilateral renal artery stenosis. Aortorenal bypass was performed successfully.- - - - - - - - - - ranking = 5.3423454189182keywords = renovascular, hypertension (Clic here for more details about this article) |
3/9. hypertension due to an aneurysm of the left renal artery in a patient with neurofibromatosis.Arterial lesions in patients with neurofibromatosis are rarely described and in most cases are stenotic. The aneurysmal changes reported in the literature are usually characterized by multiple microaneurysms due to the dysplastic lesions of the artery. We report a case of a single aneurysm of the inferior hilar branch of the left renal artery of a young female with neurofibromatosis. The patient showed hypoperfusion of the renal pole fed by this branch and was hypertensive. The aneurysm had a diameter of 4 cm and showed the histological findings typical of dysplastic lesions of neurofibromatosis. The hypertension and the renal pole hypoperfusion recovered after surgical excision of the aneurysm and end-to-end anastomosis of the hilar branch stumps.- - - - - - - - - - ranking = 0.14285714285714keywords = hypertension (Clic here for more details about this article) |
4/9. A 4-year-old boy with neurofibromatosis and severe renovascular hypertension due to renal arterial dysplasia.A 4-year-old boy had severe hypertension, cardiac failure, and signs of neurofibromatosis. Arteriography disclosed renal artery stenosis in both kidneys with signs of ischemia, particularly in the right kidney. Because of insufficient response to antihypertensive therapy, a right-sided nephrectomy was performed. Histological examination of this kidney showed segmental stenosis in all branches of the renal artery. The vascular lesions were characterized by an intimal proliferation of spindle cells in a mucoid matrix with destruction of the internal elastic membrane frequently accompanied by loss or attenuation of the media and fibrosis of the adventitia. Occasionally, a nodular arrangement of the spindle cells at the interface between intima and media was observed. Immunohistochemical studies demonstrate a smooth-muscle cell origin for these cells.- - - - - - - - - - ranking = 18.655095961387keywords = renovascular, hypertension (Clic here for more details about this article) |
5/9. Percutaneous transluminal renal angioplasty in neurofibromatosis.A 9-year-old boy with hypertension was found to have neurofibromatosis associated with stenosis of the right renal artery. Percutaneous transluminal angioplasty (PTA) was performed. Immediately post angioplasty angiography showed that the stenosis persisted, but over the next few days his blood pressure rapidly decreased and remained well controlled even when treatment was discontinued. The captopril stimulation test, performed after PTA, confirmed the return of plasma renin activity to normal values. A digital subtraction aortogram, performed 2.5 years after PTA, was unchanged. His blood pressure remained persistently normal, without anti-hypertensive agents. Based on these results, PTA is suggested as the first step in correcting renal artery stenosis due to neurofibromatosis. A complete anatomical resolution of the stenosis is probably not required since slight improvements in the renal artery lumen may be accompanied by important functional improvement.- - - - - - - - - - ranking = 0.14285714285714keywords = hypertension (Clic here for more details about this article) |
6/9. Arterial stents in the management of neurofibromatosis and renovascular hypertension in a pediatric patient: case report of a new treatment modality.A 17-year-old male with neurofibromatosis presented with severe hypertension secondary to an abdominal aortic coarctation and bilateral renal artery stenoses. Despite previous surgical bypass grafts and aggressive medical management, including treatment with diuretics, beta-blocker, calcium channel blocker, angiotensin converting enzyme (ACE) inhibitors, and direct vasodilator agents, severe hypertension persisted. Following intravascular stent placement in the aorta and both renal arteries, blood pressures normalized.- - - - - - - - - - ranking = 18.797953104244keywords = renovascular, hypertension (Clic here for more details about this article) |
7/9. Neurofibromatosis and arterial aneurysms.Neurofibromatosis (NF) is a common autosomal dominant disease characterized by the development of hamartomatous or neoplastic lesions due to the proliferation of neural crest cells. An association of aneurysmal arterial lesions with NF, which may have catastrophic complications, has been rarely reported. Our recent experiences with the diagnosis and management of three male NF patients with aneurysms is described. A 19-year-old-man with refractory hypertension due to unilateral, complex, unreconstructable renal artery aneurysms was successfully treated by nephrectomy. Histopathology demonstrated intramural renal artery Schwann cell proliferation. A 44-year-old patient underwent ligation of a ruptured superior mesenteric artery aneurysm. Finally, a femoral-popliteal artery saphenous vein bypass graft with aneurysm exclusion was performed in a 58-year-old-man with a 3.5 cm symptomatic popliteal artery aneurysm. In NF, the underlying pathology in large arteries is intramuscular Schwann cell proliferation with secondary fibrosis. Mesodermal dysplasia may affect small arteries resulting in stenosis, post-stenotic dilatation, or aneurysmal degeneration. Clinicians should be aware of the unusual association of NF with aneurysms, particularly the occult development of visceral and renal artery aneurysms. These lesions are subject to sudden rupture with potentially devastating consequences, and they mandate a high index of suspicion in NF patients.- - - - - - - - - - ranking = 0.14285714285714keywords = hypertension (Clic here for more details about this article) |
8/9. Neurofibromatosis complicated with XXX syndrome and renovascular hypertension.A 25-year-old woman with neurofibromatosis was admitted to our hospital for evaluation of hypertension. When she was 6 years old, she was diagnosed as having neurofibromatosis and XXX syndrome because of multiple cafe-au-lait spots, neurofibromas of the skin and mental retardation. Chromosome analysis revealed that her karyotype was 46, XX/47, XXX. Renal arteriography disclosed aneurysmal change and stenosis of the right renal artery. After right-side nephrectomy and aneurysmectomy, the kidney was autotransplanted in the left iliac fossa. Surgical procedure resulted in marked amelioration of the hypertension without medical treatment. Thus, aortorenal bypass and renal autotransplantation have emerged as the preferred revascularization operations. This is the first report of a chromosomal linkage between neurofibromatosis which is thought to be an autosomal dominant disease and the XXX syndrome.- - - - - - - - - - ranking = 18.797953104244keywords = renovascular, hypertension (Clic here for more details about this article) |
9/9. A case of renovascular hypertension associated with neurofibromatosis.We report a case of renovascular hypertension associated with neurofibromatosis complicated by moderate proteinuria. A 16-year-old female was admitted to Kensei General Hospital with a complaint of headache and a blood pressure of 230/120 mm Hg. She was referred to us for further evaluation of the hypertension. On examination, cafe-au-lait spots were seen over her extremities and flank, and a bruit was heard in the right upper abdomen. The urinary protein excretion was 2.1 g/day. The plasma renin activity (PRA) and plasma aldosterone concentration were high, but the levels of catecholamines were normal. The renogram was asymmetric and on venous sampling, the PRA in the right renal vein was 58.3 ng/ml/h and that in the left was 22.1 ng/ml/h. CT scan detected an approximately 10-mm mass in the proximal right renal artery. Arteriography disclosed severe stenosis in the right renal artery and the superior mesenteric artery. Therefore, we concluded that her hypertension resulted from stenosis of the right renal artery due to neurofibromatosis. Accordingly, she underwent an operation to reconstruct that artery. After the operation, her blood pressure and PRA normalized without administration of any anti-hypertensive drug and urinary protein disappeared.- - - - - - - - - - ranking = 23.426012808877keywords = renovascular, hypertension (Clic here for more details about this article) |