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

A 10-year-old girl had arterial hypertension, generalized neurofibromatosis, coarctation of the abdominal aorta and multiple stenoses at the origin of each renal artery. After resection of the stenotic areas and reimplantation of the renal arteries in the aorta, her arterial pressure decreased substantially. However, hypertension recurred and radiologic follow-up 4 1/2 years later showed distinct progression of the coarctation and renewed stenosis of all renal arteries at their origin. The stenotic areas showed eccentric intimal proliferation, frequently bulging into the lumen, with small nodular aggregates of smooth muscle cells and proliferation of fibrous tissue containing spindle-shaped nuclei in a palisading pattern. Hypertension associated with neurofibromatotic vascular disease has been described in 47 other patients in the literature. These patients have been young (mean age, 14 years) and predominantly male. In contrast to fibromuscular dysplasia, in which 95% of all stenoses are found in the distal two thirds of the renal arteries, in vascular neurofibromatosis more than 50% of the stenoses are found at the origin.
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PMID:Arterial hypertension and neurofibromatosis: renal artery stenosis and coarctation of abdominal aorta. 81 Feb 39

Pathological type complications associated with 46 cases of neurofibromatosis in children under 12 are reported. It is noted that in 65.2% of the cases there are mental retardation, usually serious. More than 50% (24 cases) had some type of tumoration. All were benign with the exception of a suprarenal neuroblastoma that caused arterial hypertension and histological characteristics of malignancy. Fifteen tumors were located in the optica ways, one in the mediastinum, one in the abdomen, one in the paravertebral area, one which was a craneal plexiform tumor and four of the moluscum pendulum type on the eyelids or in neighbouring regions. Twelve children suffered from some type of seizures (Salaam's spasms, tonic-clonic, myoclonic, atonic and versive). Radiological abnormalities were very frequent in the simple X rays as well as in those in which contrast medium was used. In four cases malformations of the midline were observed, three of which were non-communicating cysts of the septum pellucidum, the other agenesis of the corpus callosum. Neurofibromatosis was further seen associated iwth Bourneville's syndrome, Morquio's syndrome, Batten's type of lipofuscinosis, facial or generalized hemihypertrophia and stenosis of the aqueduct. Heredity was dominant autosomic in 16 cases, the rest being due to possible recent mutations.
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PMID:[Pathological complications in 46 cases of neurofibromatosis in children (author's transl)]. 82 74

The authors present a case of pheochromocytoma observed in a 49 year old man suffering from the cutaneous form of von Recklinghausen's disease. In this patient, they noted the constant absence of the inferior abdominal cutaneous reflex on the right side, contrasting with the briskness of the other abdominal cutaneous relexes. From this they concluded that the chromaffin tumor was very probably situated in the right adrenal region. This hypothesis was confirmed by retro-pneumoperitoneum, selective arteriography and finally surgical intervention. After having reviewed the essential medical data from the literature concerning the conditions associated with pheochromocytoma and especially phacomatosis, and having looked at the problem of hypertension related to neurofibromatosis, they make a critical study of the behaviour of the abdominal reflexes in case of pheochromocytoma and emphasize the part that it is possible to draw from this in view of its' topographic diagnosis. The present case is the fourth in which one of the authors has noted the abolition of the inferior abdominal reflex on the side corresponding to the site of the pheochromocytoma.
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PMID:[Pheochromocytoma and Recklinghausen's cutaneous neurofibromatosis. Localizing value of abdominal reflexes in a case]. 82 54

A case of generalized neurofibromatosis (von Recklinghausen's disease) associated with hypertension is presented. Adequate repair of the unilateral main artery stenosis did not result in cure of the hypertension because of diffuse neurofibromatous lesions of the smaller renal vessels. Review of the literature reveals that neurofibromatosis of the large and small renal vessels should be suspected in every hypertensive patient with these lesions below the age of eighteen. In view of the fact that vascular neurofibromatosis is a dynamic and spreading disease, surgery of the renal vessels should be reconstructive as much as possible.
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PMID:Neurofibromatosis and hypertension. 82 95

We report the case of a pregnant woman who presented with neurofibromatosis and hypertension the latter revealed by abruptio placentae. Severe hereditary hypertension was noted in the family during pregnancies that were sometimes complicated by intrauterine growth retardation, abruptio placentae and intrauterine foetal death. Thus, neurofibromatosis in a pregnant woman has a poor obstetrical outcome when it is associated with a personal or family history of hypertension. Such women must be treated with extreme care and hospitalized at the end of the third trimester. The advisability of prevention with low-dose aspirin during the first trimester is discussed.
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PMID:[Hereditary hypertension in Recklinghausen's disease during pregnancy. A family]. 841 95

Pheochromocytoma is a cause of hypertension that frequently can be cured by surgery. The aim of this paper, based on 5 cases of pheochromocytoma, is to relate our experience in diagnosis and treatment in this pathology. In four of 5 patients with pheochromocytoma we observed unusual characteristics of the disease. Association with neurofibromatosis in one case, with rheumatic mitral regurgitation in another; and in a third case the tumor was malignant. One patient had catecholamine-mediated electrocardiographic changes which disappeared with treatment. Since symptoms of adrenergic hyperactivity were present in all cases, the rise in the levels of vanilmandelic acid and urinary metanephrines were useful in confirming the diagnosis. Computed tomography and I-131 meta-benzylguanidine for radioisotopic imaging, displayed not only all tumoral masses but also bone metastases in the malignant case. During the follow-up period, from the sixth month to the fourth year after surgery, four patients were asymptomatic, and have normal urinary catecholamine metabolite levels. The patient with a malignant form of pheochromocytoma continued to show elevated catecholamines release and remained hypertensive in spite of adrenal mass resection.
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PMID:[Pheochromocytoma. Its diagnostic and therapeutic characteristics]. 134 Jul 39

In a 20 year period, from 1971 through 1991, 105 chromaffin tumours--excluding cervical ones--were operated by the same surgeon: 50 during the first 15 years and 55 during the last 5 years. Pheochromocytomas are defined as intra-adrenal chromaffin tumours, and paragangliomas--or ectopic pheochromocytomas--as of extra-adrenal location. Among those tumours, 30 were malignant (i.e. metastatic) and 75 benign. Among the 30 malignant tumours, 14 were ectopic, 2 occurred in a MEN II A setting and were bilateral, 2 were associated with liver adenoma and liver hemangioma respectively suggesting Von Hippel-Lindau syndrome, and one case was associated with a seemingly sporadic primary hyperparathyroidism. 9 out of those 30 malignancies were not associated with hypertension. Among 75 benign pheochromocytomas or paragangliomas, 10 were ectopic, 7 occurred in a MEN setting (6 type II, 1 type I). 3 patients without evidence of MEN or other neuroectodermal abnormalities presented bilateral pheochromocytoma, either synchronous (2) or metachronous (1). 7 cases occurred in a Von Hippel-Lindau syndrome (3 bilateral) and 4 in a neurofibromatosis setting (1 bilateral). 3 other cases were familial without evidence of MEN (including a case of triple tumour: bilateral and ectopic and another ectopic case). 2 other cases were associated with seemingly sporadic hyperparathyroidism. As a whole, in 34 of 75 benign pheochromocytomas or paragangliomas, the tumour was not intra-adrenal, unilateral and sporadic. Among those 75 tumours, 22 were not overtly hypertensive, including 10 out of the 41 seemingly intra-adrenal, solitary and sporadic. The pheochromocytoma, benign, intra-adrenal sporadic, hypertensive accounts for no more than 30% of the subphrenic catecholamine-secretin chromaffin tumours.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Can pheochromocytoma be considered a benign unilateral intra-adrenal sporadic hypertensive tumor ? Reflections on a series of 105 surgically treated subdiaphragmatic chromaffin tumors]. 134 86

Hypertension can complicate the course of neurofibromatosis. When it appears in adulthood (after 18 years of age) it is usually due to pheochromocytoma, but in children the cause is a stenotic lesion of the renal arteries or the suprarenal aorta. Its treatment follows the general principles used in the treatment of the more common forms of renovascular hypertension. We report four patients that developed renovascular hypertension after being diagnosed of neurofibromatosis, they had different localizations of the stenotic lesion, and the diverse types of surgical treatment are analyzed.
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PMID:[Neurofibromatosis as a cause of arterial hypertension in children]. 144 21

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.
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PMID:Hypertension due to an aneurysm of the left renal artery in a patient with neurofibromatosis. 146 87

Neurofibromatosis (NF), an autosomal dominant inherited disorder affecting multiple organ systems, is rare among pregnant women. NF in pregnancy has been reported to be complicated by maternal hypertension, fetal intrauterine growth retardation and fetal wastage. A case of pregnancy associated with neurofibromatosis, complicated by pregnancy aggravated chronic hypertension, asymmetric intrauterine growth retardation and fetal hydrocephalus is described. Possible interrelations between those pathologies are discussed.
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PMID:Fetal hydrocephalus associated with maternal neurofibromatosis. 147 23


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