Cases reported "Vascular Diseases"

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1/15. Disseminated intravascular meconium in a newborn with meconium peritonitis.

    A 3-day-old premature infant with meconium peritonitis, periventricular leukomalacia, and pulmonary hypertension died with respiratory insufficiency. An autopsy disclosed intravascular squamous cells in the lungs, brain, liver, pancreas, and kidneys. Numerous pulmonary capillaries and arterioles were occluded by squamous cells, accounting for pulmonary hypertension. brain parenchyma surrounding occluded cerebral vessels showed infarct and gliosis. A mediastinal lymph node filled with squamous cells alluded to the mechanism by which these cells from the peritoneal cavity likely entered the bloodstream--namely, via diaphragmatic pores connecting with lymphatics. Thus, disseminated intravascular meconium rarely may complicate meconium peritonitis and have devastating consequences.
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keywords = arteriole
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2/15. High flow priapism due to an arterial-lacunar fistula complicating initial veno-occlusive priapism.

    High flow or arterial priapism is rare, caused by unregulated arterial blood flow from a lacerated cavernous artery or branch entering directly into lacunar spaces, bypassing the protective, high resistance helicine arterioles and resulting in an arterial-lacunar fistula (ALF) and usually occurs following direct blunt or penetrating perineal trauma. Clinical features include delayed onset of a constant, painless, nontender erection of incomplete rigidity with potential for full rigidity with sexual stimulation. Colour duplex Doppler ultrasonography (CDU) is reliable in the diagnosis of arterial priapism. Treatment by arterial ligation, super-selective embolisation with autologous clot, gelatin sponge or microcoil, duplex guided compression, systemic or intracavernous administration of a variety of alpha-adrenergic agents or methylene blue, mechanical compression/ice packs or expectant management has been reported.
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keywords = arteriole
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3/15. Bone morphogenic protein-4 expression in vascular lesions of calciphylaxis.

    calciphylaxis is characterized by an extensive media-calcification of cutaneous and subcutaneous arterioles and capillaries. Recent studies have provided evidence that vascular calcification is a process with similarities to bone metabolism. Bone morphogenic protein-4 (BMP-4) is physiologically involved in bone development and repair. The presence of BMP-4 in atherosclerosis and in sclerotic heart valves led us to suggest that BMP-4 is also involved in calciphylaxis. A 47-year-old male patient developed end-stage renal failure due to chronic glomerulonephritis. He has had two kidney transplants with an immunosuppressive regimen consisting of cyclosporine A and steroids. He was admitted to our hospital because of an increase in serum creatinine (Cr) and he subsequently developed progressive dermal ulcerations. A skin biopsy led to the diagnosis of calciphylaxis. immunohistochemistry for BMP-4 of a skin specimen from our patient showed strong cytoplasmic immunoreactivity of intradermal cells with clear spatial association to arterioles and hair follicles. Whereas there are identified inhibitors and promoters of vascular calcification, the presence of BMP-4 has not been demonstrated in calcific uremic arteriolopathy. In contrast to atherosclerosis, BMP-4 in calciphylaxis cannot be found in vascular media, but in intradermal cells at the border of arterioles and hair follicles. Therefore, in calciphylaxis BMP-4 can play the role of a cytokine, a growth factor or a media-calcification promoter.
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keywords = arteriole
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4/15. Sclerosing vasculopathy of the central nervous system in nonelderly demented patients.

    Three nonelderly patients without hypertension whose clinical and radiologic features otherwise resembled Binswanger's subcortical arteriosclerotic encephalopathy underwent biopsy of the hyperintense periventricular lesions seen on magnetic resonance imaging. The pathologic findings of the periventricular lesions consisted of gliosis with mild rarefaction and edema of the white matter. All patients had a sclerosing vasculopathy of unknown cause, which involved numerous small vessels within the periventricular lesions. The vessels stained negatively for amyloid, amyloid precursors, desmin, vimentin, keratin, immunoglobulin, and complement. On electron microscopy, small arteries, arterioles, venules, and capillaries were characterized by swollen astrocytic foot processes surrounding the vessels; dense, perivascular collagen packing; crystalline arrays of filaments within basement membrane; giant lipid-laden lysosomes within perivascular cells; and narrowing of the vascular lumina. Similar changes were not seen in a control group of 19 patients. The pathologic features of the vessels in these cases are distinct from the vasculopathy associated with Binswanger's subcortical arteriosclerotic encephalopathy. We suggest that a spectrum of vasculopathies may be associated with dementia and periventricular hyperintense lesions on magnetic resonance imaging.
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keywords = arteriole
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5/15. Correlation of clinicopathologic findings in a patient. Congenital night blindness, branch retinal vein occlusion, cilioretinal artery, drusen of the optic nerve head, and intraretinal pigmented lesion.

    The ocular clinicopathologic features of this unique patient were congenital stationary night blindness, drusen of the optic nerve head, cilioretinal artery, intraretinal pigmented lesion, and branch retinal vein occlusion. Photocoagulation therapy led to total disappearance of the neovascular tissue, clinically and histopathologically. Histopathologic examination showed an occluded branch vein associated with a sclerotic retinal arteriole. Peripheral to the site of venous occlusion, inner ischemic retinal atrophy was present. The normal complement of rod and cone photoreceptors supports the view that the night blindness in this case was an abnormality in the neural transmission and not on a morphological basis. The pigmented intraretinal lesion proved to be a localized area of retinal and choroidal neovascularization with anastomosis and secondary retinal pigment epithelial hyperplasia. This lesion was identical to Fuchs' dot of myopia but out patient was hyperopic.
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keywords = arteriole
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6/15. Mitochondrial angiopathy in cerebral blood vessels of mitochondrial encephalomyopathy.

    We studied cerebral blood vessels of two autopsied patients with mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes (MELAS). All the main cerebral arteries in the proximal portion at the brain base and more distal portion at the cortical surface, as well as within the brain parenchyma were examined by electron microscopy. There was a striking increase in number of mitochondria in the smooth muscle and endothelial cells, which were most prominent in the pial arterioles and small arteries up to 250 micron in diameter and less frequent and severe in the larger pial arteries and intracerebral arterioles and small arteries. These vascular changes have not hitherto been described in MELAS, or in other disorders affecting blood vessels of the brain and other organs. It is suggested that the vascular changes are caused by primary mitochondrial dysfunction in the vascular smooth muscle and endothelial cells of the brain and that they constitute the pathogenic base of the brain lesions and their unusual distribution pattern in MELAS.
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keywords = arteriole
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7/15. amyloid angiopathy combined with granulomatous angiitis of the central nervous system: report on two patients.

    We report here two cases of isolated angiitis of the central nervous system associated with congophilic angiopathy. The clinical history lasted 9 months in the first patient (65 years old) and 9 years in the second patient (59 years old). It was characterized by progressive intellectual deterioration, increased protein content of the CSF and evidence of focal brain lesions in the CT scan. One patient showed chronic intracranial hypertension. Vascular lesions were limited to the brain and were characterized by granulomatous and necrotizing angiitis of the small leptomeningeal and intracortical vessels. amyloid deposits were present in large amounts along vascular segments showing vasculitis, in foreign body giant cells, in plaque-like structures surrounding diseased perforating arterioles, along cortical microvessels and in many neuritic plaques. Close proximity and topographic overlap of vasculitis and amyloid changes suggest a possible pathogenetic relationship.
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keywords = arteriole
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8/15. Unusual causes of arterial insufficiency.

    Most patients with vascular insufficiency in the legs suffer from atherosclerosis. Thus, it is easy to miss the diagnosis in occasional patients with disease due to other causes. Mechanical and functional disorders which affect major arteries or arterioles are described.
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keywords = arteriole
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9/15. Microangiopathic hemolytic anemia, renal failure, and noncardiogenic pulmonary edema: a chemotherapy-induced syndrome.

    Following gastrectomy for locally advanced adenocarcinomas, three patients developed microangiopathic hemolytic anemia and renal failure shortly after completing courses of adjuvant chemotherapy with mitomycin and 5-FU. These complications progressed despite cessation of chemotherapy, and all three patients died of noncardiogenic pulmonary edema precipitated in two cases by blood transfusions. At autopsy, two patients had no residual carcinoma and all had a diffuse microangiopathy involving mainly the kidneys and lungs. There was intimal hyperplasia of many arterioles sometimes associated with complete occlusion of the lumen, prominent nuclear atypia in many capillary cells, and numerous capillary fibrin thrombi. Direct immunofluorescence studies revealed extensive fibrinogen-fibrin deposits in the vascular lesions. Chemotherapy-induced microangiopathic hemolytic anemia and renal failure may predispose patients to fatal episodes of noncardiogenic pulmonary edema that can be triggered by blood transfusions.
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keywords = arteriole
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10/15. Unusual vascular tumour of the scalp in association with lymphoid aggregates: a variant of angiolymphoid hyperplasia?

    A 51-year-old mason presented with a large tumour on his scalp which had developed over the previous 2 years. Histological examination showed the presence of large vessels with muscular coats some of which appeared to be venules and others arterioles. They had markedly swollen cuboidal endothelial cells and a surrounding mononuclear cell infiltrate which in areas formed lymphoid follicles some of which had germinal centers. There was no evidence of tissue or blood eosinophilia. The unusual clinical and histological features of this case are emphasized and the nosology of this rare condition is discussed.
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keywords = arteriole
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