Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0020538 (hypertension)
 170,190 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We present two patients with renovascular hypertension in association with neurofibromatosis. In both cases the diagnosis was established by angiography. The first patient suffered occlusion of the right renal artery combined with intrarenal arterial lesions. Treatment was by autotransplantation. The second patient suffered bilateral intrarenal arterial lesions alone and was treated medically. In both patients, control of the hypertension was established. We discuss the aetiology of renovascular hypertension in neurofibromatosis and consider its treatment.
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PMID:Renovascular hypertension in neurofibromatosis. 250 38

Having seen a personal case and having reviewed the literature, we clarify the details when neurofibromatosis and pregnancy occur together. Few cases of this combination of conditions have been reported. The complications can be serious and have led some authors to suggest that the pregnancy should be terminated in every case and that the patient should be sterilised. Sometimes the only risk is hypertension of pregnancy, which is what occurred in our case. Its progress was benign and according to the literature this seems to be the most usual outcome. All the same, because it is possible that complications can occur in any case of neurofibromatosis in pregnancy, pregnant women with neurofibromatosis should be placed in a high-risk group which has to be watched very carefully.
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PMID:[Von Recklinghausen's neurofibromatosis and pregnancy. Apropos of a case]. 250 43

An 11-year-old boy with slowly progressive gangrene caused by vasculopathy similar to that of neurofibromatosis (NF) type 1 (NF I; von Recklinghausen disease [NFvR]) and a newborn girl with idiopathic gangrene with vascular changes resembling those of NFvR prompted the analysis of all 105 propositi with NF (NF I and NF II) evaluated between January 2, 1982, and December 31, 1986, at the genetics clinic of University of South Florida. They were analyzed for renal hypertension, symptomatic ischemia, and known vascular changes. One additional 27-month-old boy with NFvR was found to have extensive vascular changes with renal hypertension. The vasculopathy indicated asymmetric over/undergrowth of cellular and extracellular components of the vascular wall and implied dysregulation of the paracrine growth mechanism. Immunocytochemical studies of affected vessels were done only in the 11-year-old boy and showed positive neuron-specific enolase, S-100 protein, and glial fibrillary acidic protein (GFAP) reactions indicative of Schwann cell involvement. The vascular changes in children with NFvR are mostly asymptomatic; however hypertension secondary to renal artery stenosis and/or Moya-moya disease have been reported infrequently. Our patients with vasculopathies provoked thoughts in regard to the so-called vascular NF, its place in current NF nomenclature and classification, relationship to fibromuscular dysplasia (FMD), and possible role in infantile gangrene.
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PMID:"Vascular neurofibromatosis" and infantile gangrene. 251 May 17

It is reported on an abnormally prolonged action of vecuronium in a female patient with known neurofibromatosis (Morbus von Recklinghausen). In this female patient an increased sensitivity to vecuronium (0.06 mg/kg) was observed during anaesthesia for Caesarean section. A 100% block lasted 50 minutes and the neuromuscular recovery from 75% block to 25% block amounted to 25 minutes. This prolonged neuromuscular block can also be due to overdosing, relative hypovolaemia during arterial hypertension and the previous application of suxamethonium.
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PMID:[Prolonged action of vecuronium in neurofibromatosis (von Recklinghausen's disease)]. 256 40

Monozygotic twins sisters with optic glioma "in mirror image" (one with involvement of the left optic nerve and the other with the right optic nerve) and hydrocephalus secondary to progressive stenosis of the aqueduct have been found in a series of 128 cases below 14 years of age with neurofibromatosis. The optic glioma was diagnosed in each of the twins at 2 years of age. In one twin the tumor involved only the optic nerve but in the other the glioma affected the optic nerve and spread to the homolateral zone of the optic chiasm. First symptoms of hydrocephalus appeared at 8 years and 11 years of age respectively but ventriculo-peritoneal shunting procedures were performed to relieve intracranial hypertension at 11 years and 15 years of age respectively. At 2 years of age both twins had pneumoencephalography which demonstrated normal air passage through the aqueduct and cerebral ventricles of normal size and morphology. Posterior studies with CT-scan demonstrated progressive obstruction of the aqueduct with very slow progression of the hydrocephalus in each twin, although it was not observed simultaneously. The increased intracranial pressure was tolerated for many years in each twin without obvious symptoms which could be attributed to the slow progression of the aqueduct obstruction.
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PMID:Optic glioma with progressive occlusion of the aqueduct of Sylvius in monozygotic twins with neurofibromatosis. 283 13

We report a case of adrenal phaeochromocytoma with the stigmata of neurofibromatosis, who presented with acute hypotension. The patient later had episodes of hypertension alternating with hypotension, associated with electrocardiographic changes simulating myocardial infarction, and finally a completed stroke. The circumstances illustrate the problem of early recognition of the protean clinical effects of an excess of circulating adrenal medullary hormones. They also emphasise the need to consider phaeochromocytoma as a differential diagnosis of apparent clinical shock.
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PMID:Adrenal phaeochromocytoma and neurofibromatosis presenting with hypotension. 287 1

A 12 year old girl with severe arterial hypertension was found to have neurofibromatosis associated bilateral stenoses of the main renal arteries and elevated plasma renin activity in the right main renal vein. Antihypertensive treatment was unable to normalize blood pressure. PTA of the right renal artery from a left axillary approach resulted in normalization of blood pressure and peripheral plasma renin activity. PTA seems to be an effective and safe method for treatment even of complicated forms of renal artery stenosis.
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PMID:Treatment of neurofibromatosis associated renal artery stenosis with hypertension by percutaneous transluminal angioplasty. 294 78

In the past two decades, 853 children and adolescents have been evaluated for elevated BP as inpatients and outpatients by the hypertension service at Children's Hospital Medical Center, Cincinnati. Most children with hypertension secondary to coarctation of the aorta and all children with glomerulonephritis are managed by other services and are not included in that total. In a retrospective study, 27 children (3%) were identified as having hypertension secondary to intrinsic renal arterial stenosis. The age at presentation ranged from 5 months to 20 years. The mean BP at that time was 171.6/114.2 mm Hg. Ten categories of causes were identified, including fibromuscular dysplasia, arteritis, Williams syndrome, neurofibromatosis, congenital malformations, blunt abdominal trauma, surgical vascular trauma-irradiation, thrombosis, congenital rubella syndrome, and unknown. Overall, symptoms were not common. However, findings of end-organ response, such as left ventricular hypertrophy and retinal vascular abnormalities were prevalent. This is not surprising given the mean BP level at presentation. Physical examination, laboratory tests, and radiologic evaluations (exclusive of renal angiography) were not useful in detecting or identifying the location and extent of the renovascular lesions. Fourteen patients were treated surgically, and 13 were managed medically. The outcome was variable for both treatment modalities. The management of renovascular hypertension in children must be individualized depending on the cause, location, and severity of the lesion, as well as the size of the child. Some forms of renovascular pathology, particularly the arteritides, may resolve spontaneously, and children with these entities should have their BP treated medically until the inflammatory process has subsided.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Clinical spectrum of intrinsic renovascular hypertension in children. 295 4

Percutaneous transluminal angioplasty (PTA) has been developed over the past 8 years as an alternative to reconstructive surgery for renovascular hypertension. We report three cases and review the use of PTA in children with renal artery stenosis. At least 37 cases of PTA have been reported in patients whose ages ranged from 1.3 to 17 years (mean 10 years). Of these, 10 had fibromuscular dysplasia; 13 unspecified unilateral renal artery stenosis; 4 bilateral stenosis; 4 neurofibromatosis; 4 renal transplant; 1 atherosclerosis; and 1 postsurgical stenosis. Nine of 10 patients with fibromuscular dysplasia were cured and 3 of 4 with renal transplant arterial stenosis were cured or improved. There were 11 failures of PTA, including all 4 patients with neurofibromatosis and 1 with transplant arterial stenosis. We conclude that PTA is the treatment of choice for children with hypertension due to fibromuscular dysplasia and should be attempted for stenosis of the transplanted renal artery. Other lesions resulting in renal artery stenosis may not be as amenable to dilation and should be considered on an individual basis.
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PMID:Percutaneous transluminal angioplasty for renovascular hypertension in children. 297 67

To determine the long-term prognosis for hypertension control, mortality, renal function, and maintenance of renal blood flow in patients operated on to control renovascular hypertension, we studied 60 patients managed surgically between 1969 and 1984. Thirty-six patients had atherosclerotic disease, 22 had fibromuscular dysplasia, one had neurofibromatosis, and one had a combination of atherosclerosis and pyelonephritis. We confined the analysis to the 58 patients with pure atherosclerosis or fibromuscular dysplasia. In the atherosclerosis group 14 patients died and the results of hypertension control in the remaining 22 were classified as cured, three (14%); improved, 15 (68%); failed, one (5%); and unknown, three (14%). In the fibromuscular dysplasia group one patient died and results of hypertension control in the remaining 21 patients were (1) cured, 10 (48%); improved, 10 (48%); and failed, one (5%). The 5- and 10-year survival rates were 79% and 40%, respectively, for the atherosclerosis group and 95% and 89%, respectively, for the fibromuscular dysplasia group. Renal function was well maintained for patients in both groups. The mean serum creatinine value was 1.4 mg/dl in the atherosclerosis group and 1.0 mg/dl in the fibromuscular dysplasia group. To evaluate the effect of operation on the maintenance of renal blood flow we compared the blood flow of the operated and unoperated sides in patients who had a unilateral operation and had a second kidney for comparison. Eight of these patients had scans in each of the two groups.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Long-term prognosis of surgical treatment of renovascular hypertension: a fifteen-year experience. 308 37


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