Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0004153 (atherosclerosis)
 77,401 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

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

Therapeutic results in 102 hypertensive patients were evaluated after either renal artery percutaneous transluminal angioplasty (PTA) or surgical bypass procedures for renovascular hypertension. A minimum of 6 months of follow-up was accepted to evaluate therapeutic success or failure. Renal angioplasty had a beneficial result in each of 13 patients with fibromuscular dysplasia and in 10 (83%) of 12 patients with atherosclerotic lesions that did not involve the origin of the renal artery. Although surgery was also beneficial in each of six patients with fibromuscular dysplasia, it helped only five of 10 patients with atherosclerosis of the renal artery. Angioplasty results were similar to surgical results for atherosclerotic lesions that involved the origin of the renal artery. Angioplasty was unsuccessful in two cases of neurofibromatosis because of the firm nature of the lesions, where a bypass procedure was successful in one case. Major complications were more common in surgical cases than in angioplasty. PTA is recommended for all renal artery lesions; surgery should be reserved for failed PTA or recurrent renal artery stenosis after PTA.
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PMID:Percutaneous transluminal angioplasty vs. surgery for renovascular hypertension. 315 91

Whereas the information on the subject of arterial status is sketchy and haphazard with respect to any one genetic disorder, the number of these diseases would have precluded the provision of a critical review within the scope of this presentation. Thus, it was deemed more meaningful to approach the subject selectively. A brief summary was provided on the nature of the arterial wall and its involvement in genetic diseases either as a primary target or a secondarily affected organ, and on "affinity" of various genetic disorders for a type (elastic, muscular, or smallest), segment (proximal, distal), and layer (intimal, medial, adventitial) of the arterial tree or the arterial wall, respectively. Genetic diseases may affect arteries by "causing" (a) congenital malformations, (b) alteration of the arterial "makeup" without necessarily producing definable lesions, and (c) modification of a nongenetic arterial disease (e.g., atherosclerosis), or by "producing" (d) arterial lesions that are characteristic of (even specific for?) a given genetic disorder. A few examples were selected to illustrate (b) (tuberous sclerosis; infantile GM1-gangliosidosis), (c) Wolman's disease; familial hyperlipoproteinemias), and (d) [Hurler's disease, neurofibromatosis; Ehlers-Danlos syndrome (type IV)]. Whenever available, the results of electron microscopic studies carried out in our laboratories were included. Some of these have not been reported in the literature to date. The need for a coordinated multidisciplinary approach to the study of genetic diseases in general is stressed in closing.
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PMID:Arterial involvement in genetic diseases. 333 42

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

1. We have treated 25 patients with renovascular hypertension with balloon catheter dilatation. 2. In 17 patients, surgical treatment was thought to be either risky or difficult due to the presence of a serious medical condition or because of the peripheral location of the lesion. 3. Seventeen patients had atherosclerotic lesions, six patients had fibromuscular dysplasia and one patient had neurofibromatosis and one had stricture of an aorto-renal bypass graft. 4. Of the total group, six in the atherosclerotic group, three patients with fibromuscular dysplasia and the one with neurofibromatosis are normotensive without medications. 5. Five patients with atherosclerosis, two patients with fibromuscular dysplasia and the patient with aorto-renal bypass graft are either normotensive or have reasonable control of blood pressure with medication. 6. Four technical and treatment failures and three major medical complications were encountered. Recurrence of stenosis occurred in eight patients but none of these was in the fibromuscular dysplasia group.
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PMID:Transluminal balloon dilatation of renal artery stenosis causing hypertension: 18 months' experience. 744 4

Ischemic strokes may have distinct aetiologies, including several different intrinsic arterial pathological disorders. The diagnosis and understanding of these arterial diseases is critical for the correct management of stroke as different treatment approaches are undertaken according to the aetiology. Atherosclerosis is by far the most common arterial disease among adults, and other pathological processes include arterial dissection, small vessel disease, inflammatory and non-inflammatory vasculopathy and vasomotor disorders. In children, there are several vasculopathies responsible for vaso-occlusive disease such as sickle-cell anemia, acute regressive angiopathy and Moya-Moya disease, neurofibromatosis, dissections, vasculitis associated with intracranial and systemic infections. An overview of the major carotid and vertebral pathological diseases responsible for ischemic stroke in adults and children, highlighting the accuracy of the different imaging modalities for its diagnosis and the imaging appearance of these diseases, is given.
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PMID:Ischemic stroke: carotid and vertebral artery disease. 1565 89

Multiple aneurysms are clinically common in population aged over sixty and are caused mainly by atherosclerosis. When occurring in young population other etiologies such as trauma, infections, Bechet's disease, Marfan syndrome, neurofibromatosis or inflammatory disease are responsible for the development of arterial aneurysms. A rare case of multiple aneurysms in a 40-year-old man, affecting the infrarenal part of abdominal aorta, both iliac arteries, common femoral arteries, left femoral superficial and popliteal arteries on, both legs, is reported. The underlying pathology was progressive atherosclerosis, favored by familial hyperlipidemia and excessive cigarette smoking.
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PMID:A rare case of multiple aneurysms in a young patient. 1661 4

Although vascular involvement in type 1 neurofibromatosis (NF1) is rare, it may lead to renal artery stenosis and renovascular hypertension (RVH). RVH may be treated using antihypertensive drugs, percutaneous transluminal renal angioplasty (PTRA), surgical reconstruction of the renal artery, or nefrectomy. In NF1 the results of PTRA are less predictable than in cases of fibromuscular dysplasia and atherosclerosis. We report a case of RVH associated with NF1. Despite administration of multiple antihypertensive drugs blood pressure remained uncontrolled. Selective left renal arteriography demonstrated two consecutive high-grade stenotic lesions with post-stenotic aneurysmal dilatation treated successfully with balloon dilatation. During the ensuing 2 year follow up complete normalization of blood pressure was observed. This case illustrates that endovascular therapy may be beneficial and should be considered a reasonable first option in these patients. However vascular involvement in NF may be progressive and therefore always requires continuing follow up.
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PMID:Renovascular hypertension associated with neurofibromatosis: a case report. 1745 Nov 48

Magicin, a protein that we isolated earlier as an interactor of the neurofibromatosis 2 protein merlin, was independently identified as MED28, a subunit of the mammalian Mediator complex. Mediator complex is an evolutionarily conserved transcriptional cofactor, which plays an essential role in positive and negative gene regulation. Distinct Mediator subunit composition is thought to contribute to gene regulation specificity based on the interaction of specific subunits with subsets of transcription factors. Here we report that down-regulation of Med28 expression in NIH3T3 cells results in a significant induction of several genes associated with smooth muscle cell (SMC) differentiation. Conversely, overexpression of MED28 represses expression of SMC genes, in concordance with our knockdown data. More importantly, multipotent mesenchymal-derived murine precursors can transdifferentiate into SMCs when Med28 is down-regulated. Our data also show that Med28 functions as a negative regulator of SMC differentiation in concert with other Mediator subunits including Med6, Med8, and Med18 within the Mediator head module. Our results provide strong evidence that MED28 may function as a scaffolding protein by maintaining the stability of a submodule within the head module and that components of this submodule act together in a gene regulatory program to suppress SMC differentiation. The results presented here demonstrate for the first time that the mammalian Mediator subunit MED28 functions as a repressor of SMC differentiation, which could have implications for disorders associated with abnormalities in SMC growth and differentiation, including atherosclerosis, asthma, hypertension, and smooth muscle tumors.
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PMID:Mediator subunit MED28 (Magicin) is a repressor of smooth muscle cell differentiation. 1784 60


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