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Patients with NF-I have a wide spectrum of vascular abnormalities, most notably aneurysms or stenoses of the aortic, renal, and mesenteric circulation. Operative treatment of symptomatic patients with vascular lesions or large aneurysms is safe, effective, and durable.

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Autopsy studies suggest that splanchnic artery aneurysms may be more frequent than abdominal aortic aneurysms. These aneurysms are important to recognize because up to 25% may be complicated by rupture, and the mortality rate after rupture is between 25% and 70%. However, little is known about the natural history and clinical presentation of splanchnic artery aneurysms. Splenic artery aneurysms are the most common of the splanchnic artery aneurysms; multiple aneurysms are present in approximately one third of patients. Hepatic artery pseudoaneurysms are more common than true aneurysms because of increasing numbers of hepatobiliary interventional procedures. The diagnosis of splanchnic artery aneurysm should be considered in any patient with abdominal pain, a pulsatile mass, or an abdominal bruit with or without associated bleeding. However, most aneurysms are asymptomatic and are detected incidentally on imaging studies. Treatment, which can be either surgical or interventional radiology-based, should be considered in all patients with symptoms related to the aneurysms, if the aneurysm is more than 2 cm in diameter, if the patient is pregnant, or if there is demonstrated growth of the aneurysm.

GCA is not a single entity but includes several variants of disease. Large-vessel GCA produces a distinct spectrum of clinical manifestations and often occurs without involvement of the cranial arteries. Large-vessel GCA requires a different approach to the diagnosis and probably also to treatment.

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The restenosis or occlusion that frequently follows balloon angioplasty is poorly understood. Thus, the pathophysiological response to angioplasty of the common carotid artery in 38 heparinized normal pigs was investigated by quantification of the 111In-labeled platelet deposition and histological and electron microscopic examination from 1 hour to 60 days after angioplasty. At 1 hour, the following findings were noted: complete endothelial denudation in all arteries, marked platelet deposition (44.7 +/- 20.7 X 10(6)/cm2), mural thrombus in seven of 10 pigs, and a medial tear extending through the internal elastic lamina in nine of 18 arteries. All nine arteries with tears had associated mural thrombus and severe platelet deposition (76 X 10(6)/cm2); in contrast, the nine arteries without a tear had no mural thrombus and much lower platelet deposition (6 X 10(6)/cm2). Necrosis of medial smooth muscle cells was evident at 24 hours. Platelet deposition remained high at 24 hours (40.5 +/- 20.6 X 10(6)/cm2), but was markedly reduced at 4 days (4.4 +/- 1.5 X 10(6)/cm2), coincident with partial regrowth of endothelium or periluminal lining cells. No significant platelet deposition was noted at 7 days, when the endothelial cell type of regrowth was largely complete. Intimal proliferation of smooth muscle cells was mild and patchy at 7 days, significantly greater and more uniform at 14 days, and unchanged at 30 and 60 days after angioplasty. Complete thrombotic occlusion occurred in four (11%) of the 38 pigs. A significant stenosis present at 30 days after angioplasty was shown by histological examination to be due to organization of mural thrombus.(ABSTRACT TRUNCATED AT 250 WORDS)