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1/35. Microvascular endothelial abnormality in skeletal muscle from a patient with gastric cancer without dermatomyositis.

    We found a microvascular endothelial abnormality in a biopsy specimen from the gastrocnemius muscle of a patient with gastric cancer, who had severe myalgia and angialgia in the calf region with the symptoms of thrombophlebitis. There were no definite findings of inflammatory myopathy in histochemical and immunohistochemical studies. Electron microscopic examination revealed the accumulation of abnormal mitochondria in the subsarcolemmal area, and a fair number of degenerating capillaries. Immunohistochemical analysis of procoagulant or anticoagulant factors revealed marked reduction of thrombomodulin (TM) expression on small vessels and capillaries. Although a reduction of TM on small vessels has been observed around perifascicular atrophic fibers in patients with dermatomyositis, histochemical findings of the present patient showed no perifascicular atrophy or severely degenerating fibers. These pathological findings in the patient may be related to a malignant neoplasm and may be one of the causes of disseminated intravascular coagulation (DIC), which is the main complication of malignant neoplasms. Further studies are necessary to determine whether the reduction of TM on the small vessels and capillaries in skeletal muscle is a predictor of some severe condition such as DIC or a rare pathological finding in some special condition such as scirrhous carcinoma with thrombophlebitis.
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2/35. Post-operative renal failure caused by disseminated intravascular coagulation.

    A 22-year-old man suffered a stab wound of the femoral artery and vein. This was followed by disseminated intravascular coagulation. Renal failure then occurred presumably due to fibrin deposition in the small vessels of the kidney. The D.I.C. was successfully treated with heparin and the renal failure with peritoneal dialysis. It is suggested that D.I.C. and consequent alterations in regional blood flow following trauma are not uncommon, and search should be made for these phenomena in every case of major trauma.
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3/35. neurologic manifestations of intravascular coagulation in patients with cancer. A clinicopathologic analysis of 12 cases.

    Among 1,459 autopsied patients with cancer, 12 had multifocal infarcts of the brain that appeared to be caused by intravascular coagulation. Most of these patients were women with leukemia or lymphoma, and all had a clinical course in which neurologic signs and symptoms were prominent. All had evidence of generalized brain disease (delirium and stupor or coma), and several also had focal brain disease (focal seizures, hemiparesis). All patients had laboratory evidence of coagulation abnormalities, although these were often not severe when neurologic symptoms began. Pathologically, there were multifocal hemorrhagic or ischemic infarcts in the distribution of several cerebral vessels, without a systemic source for cerebral emboli. fibrin thrombi were identified in cerebral vessels and in vessels of several other organs. The clinical findings fit the pathologic picture, and in most instances the correct diagnosis might have been made earlier had it been considered.
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4/35. serum tryptase analysis in a woman with amniotic fluid embolism. A case report.

    BACKGROUND: Recent studies have noted a striking similarity between amniotic fluid embolism (AFE) and anaphylaxis. serum tryptase levels may therefore serve as a marker of mast cell degranulation in AFE cases. CASE: A 40-year-old woman, gravida 6, para 4, experienced the acute onset of facial erythema, eclampsia-type seizures, severe hypoxia, cardiac arrest and disseminated intravascular coagulation while in early active labor. The patient was declared dead 37 minutes after the onset of resuscitative efforts. At autopsy, fetal squames were found within the pulmonary tree, uterine blood vessels and brain. A peripheral venous blood specimen, obtained approximately one and a half hours postmortem, revealed a tryptase level of 4.7 ng/mL (normal, < 1). CONCLUSION: An elevated serum tryptase level, in conjunction with our patient's clinical history, adds further supporting evidence to the concept of AFE as an anaphylactoid syndrome of pregnancy.
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5/35. Recurrent nonocclusive mesenteric ischemia after resection of iliac artery aneurysm.

    A case of recurrent nonocclusive mesenteric ischemia in a patient with isolated internal iliac artery aneurysm penetrating the sigmoid colon is described. On the day after the aneurysm and the sigmoid colon had been resected, the patient developed necrosis of the left hemicolon. Fourteen and nineteen days after left hemicolectomy, massive intestinal bleedings occurred, requiring ileectomy. On the basis of operative findings of good pulsation of visceral arterial branches; angiography showing patent mesenteric vessels with some spasms; and pathological findings suggesting mesenteric ischemia, these ischemic events were diagnosed as nonocclusive mesenteric ischemia. Low-output syndrome induced by massive intestinal bleeding and atrial fibrillation and sepsis were responsible for the establishment of the nonocclusive mesenteric ischemia. Development of disseminated intravascular coagulation and continuous administration of diuretics for acute renal failure seemed to have further perturbed the mesenteric circulation. The patient died of subsequent multiple organ failure 4 months after the first operation. We should pay more attention to nonocclusive mesenteric ischemia in patients with mesenteric ischemia, and strict circulatory management during the perioperative period is essential in these patients.
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6/35. adult purpura fulminans associated with staphylococcal infection and administration of colony-stimulating factors.

    purpura fulminans (PF) is a rare syndrome of progressive haemorragic necrosis due to disseminated intravascular coagulation (DIC) and dermal vascular thrombosis leading to purpura and tissue necrosis. PF is more often associated with either a benign infection or a severe sepsis. Rarely, it has been related to drug intake. We report the case of a 24-year-old female patient who suffered from staphylococcal sepsis and pancytopenia, for which she was treated with antibiotics, granulocyte-colony stimulating factor (G-CSF) and granulocyte/macrophage CSF (GM-CSF). Two days after the last GM-CSF dose, she developed widespread necrotic plaques with erythematous borders and purpura in the breast, arms and legs. Coagulation tests indicated DIC and a skin biopsy showed fibrin thrombi in the superficial dermal vessels. The patient totally recovered after removal of the necrotic tissues and application of skin autografts. Although staphylococcal infection was most probably involved in the development of PF, a role of CSF cannot be excluded in this case.
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7/35. Low utilisation of unactivated protein c in a patient with meningococcal septic shock and disseminated intravascular coagulation.

    BACKGROUND: Activated protein c has recently been shown in a multicentre trial to significantly reduce mortality in patients with septic shock. There are also some case reports and minor studies demonstrating promising results with the unactivated form of protein c. However, in children with severe meningococcal infection, skin biopsies have demonstrated low expression of endothelial thrombomodulin and protein c receptors, suggesting low protein c activation capacity in severe meningococcal sepsis. methods: A patient with meningococcal septic shock was treated with two doses of the unactivated form of protein c, the first during intense activation of the coagulation system and the second during a phase of low grade or no activation. Repeated plasma samples were analysed for protein c concentration, which made it possible to compare pharmacokinetics and half-lives of the two administrations. A shorter half-life during intense coagulation was expected if there was an activation and consumption of the protein c administered. RESULTS: The calculated half-lives of protein c during intense and low grade activation were 32 h and 19 h, respectively, a magnitude similar to that reported in protein c-deficient patients without infection. CONCLUSION: The result indicates that whole body utilisation of the unactivated protein c was low. Endothelial impairment of protein c activation does not seem to be restricted to the skin vessels only.
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8/35. Acute skin and fat necrosis during sepsis in a patient with chronic renal failure and subcutaneous arterial calcification.

    Calcification of small subcutaneous arteries and arterioles is commonly found in patients with chronic renal failure (CRF), but the syndrome of acute ischemic necrosis of the skin and subcutaneous fat supplied by these vessels is relatively uncommon. The necrosis occurs during dialysis and after successful renal transplantation, and it is often fatal. Occlusion of the calcified arteries and associated microvessels by thrombi is reported infrequently, but it is relevant to the necrosis. However, the pathogenesis remains enigmatic. In the patient described here, who had CRF, bacteremia, and laboratory evidence of disseminated intravascular coagulation (DIC), the distribution of thrombi and necrosis was mainly that of the calcified arteries which, therefore, probably played a role in the localization of the thrombi. An increased susceptibility of the endothelium of calcified vessels to the procoagulant effects of sepsis may be a contributing factor.
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9/35. Symmetrical peripheral gangrene: association with noradrenaline administration.

    The syndrome of symmetrical peripheral gangrene is characterised by distal ischaemic damage in two or more extremities, without large vessel obstruction. Four patients with bilateral pedal ischaemia are described and their haemodynamic profiles presented. In all four cases the syndrome developed in association with noradrenaline administration, sepsis and DIC, despite a high cardiac output and a low calculated systemic vascular resistance index. Early treatment with epoprostenol was instituted in the final case and was successful.
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10/35. Disseminated carcinomatosis of bone marrow from submucosal carcinoma in adenoma of the rectum.

    A 62-year-old man was admitted because of paresis of the legs and a bleeding tendency. He was diagnosed as metastatic bone cancer with disseminated intravascular coagulation (DIC). In spite of treatment, his general condition progressively deteriorated and he died of respiratory failure 13 days later. autopsy revealed a carcinoma in adenoma in the rectum. Although the depth of cancer invasion was confined to the submucosal layer, disseminated carcinomatosis of the bone marrow and tumor emboli in blood vessels of the lung were present.
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