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Primary sarcomas that arise from major blood vessels are exceedingly rare, and some of the published cases have been autopsy reports. Most patients are adults. We report a case of pulmonary artery sarcoma in a 77-year-old man who presented with acute onset of dyspnea. Magnetic resonance imaging of the chest revealed a large mass within the pulmonary trunk and its main branches. Because massive pulmonary embolism was suspected, both anticoagulant and thrombolytic therapies were initiated. The patient responded poorly to these therapies, which then necessitated resection of both the mass and the pulmonary valve. A bioprosthetic porcine valve replaced the native valve, and we reconstructed the right ventricular outflow tract with a Dacron patch. Histopathologic examination revealed a high-grade sarcoma with focal myogenic and chondrogenic differentiation. The patient tolerated the procedure well and was discharged from the hospital on postoperative day 7. He was subsequently treated with chemotherapy and radiation and continued to show no evidence of disease.The diagnosis of pulmonary artery sarcoma should be suspected in patients who present with manifestations of pulmonary embolism, especially when there is no evidence of deep venous thrombosis and poor response to anticoagulant therapy. Multimodal therapy can provide prolonged survival.  相似文献   

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We describe the case of a 60-year-old woman who presented with pulmonary artery sarcoma, a very rare tumor of the cardiovascular system. Her tumor was initially misdiagnosed as chronic pulmonary thromboembolism, and she underwent pulmonary endarterectomy.Early diagnosis of primary pulmonary artery sarcoma is crucial. That alternative should always be considered before settling on a diagnosis of pulmonary embolism. Suspicion should be aroused by the failure of anticoagulant treatment to alleviate pulmonary perfusion abnormalities and systemic symptoms. Surgical resection of the tumor—preferably by pulmonary endarterectomy, followed by reconstruction as needed—is currently the most promising treatment for pulmonary artery sarcoma.  相似文献   

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Cryptococcosis is an invasive fungal infection (IFI), caused predominantly by Cryptococcus neoformans or Cryptococcus gattii, that affects both immunocompromised (IC) and non-IC patients. Although the most serious disease manifestation is meningoencephalitis, cryptococcal pneumonia is underdiagnosed and may disseminate to the central nervous system (CNS) and other sites depending upon host defenses and administration of appropriate antifungal therapy. The clinical presentation of pulmonary cryptococcosis varies along a spectrum from asymptomatic infection to severe pneumonia and respiratory failure, and the radiological presentation can be characterized by an array of findings, including nodules, consolidation, cavitary lesions, and a diffuse interstitial pattern. Diagnosis most often relies upon isolation of Cryptococcus from a pulmonary specimen in the appropriate clinical and radiological context. Treatment recommendations include induction therapy with an amphotericin B preparation and flucytosine for IC patients and those with severe disease and fluconazole for mild-to-moderate, localized disease. Knowledge of the pathophysiology, epidemiology, clinical presentation, and treatment of pulmonary cryptococcosis may lead to greater recognition of this underdiagnosed IFI and improved outcomes.  相似文献   

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Coccidioidomycosis refers to the spectrum of disease caused by the dimorphic fungi Coccidioides immitis and Coccidioides posadasii. Clinical manifestations vary depending upon both the extent of infection and the immune status of the host. Coccidioidomycosis has been reported to involve almost all organ systems; however, pulmonary disease is the most common clinical manifestation. The incidence of coccidioidomycosis continues to rise, and primary coccidioidal pneumonia accounts for 17 to 29% of all cases of community-acquired pneumonia in endemic regions. The majority of patients with coccidioidomycosis resolve their initial infection without sequelae; however, several patients develop complications of disease ranging in severity from complicated pulmonary coccidioidomycosis to widely disseminated disease with immediately life-threatening manifestations. This review focuses on complications of pulmonary coccidioidomycosis with an emphasis on the management of primary coccidioidal infection, solitary pulmonary nodules, pleural effusions, cavitary disease, acute respiratory distress syndrome (ARDS), miliary disease, and sepsis.  相似文献   

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Pulmonary telangiectasia   总被引:1,自引:0,他引:1  
Two patients with pulmonary telangiectasia are described. One of these is by far the oldest yet reported. The diagnosis and prognosis of this condition are discussed.  相似文献   

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The incidence and mortality of acute pulmonary embolism (PE) remain ill defined, particularly in the setting of the emergency department. However, high-risk groups can be identified based on medical conditions known to predispose patients to venous thrombosis. Recent research into the physiologic regulation of coagulation and thrombolysis reveals that recurrent venous thrombosis and PE may be caused by heritable deficiencies and abnormalities of plasma proteins. To decide among options for evaluation and treatment of patients suspected of PE, physicians combine clinical assessment with patterns observed on radionuclide ventilation-perfusion (V/Q) scans. However, the prevalence of PE among patients with "low probability" V/Q scans suggests that current physician behavior may be imprudent. Heparin anticoagulation continues to be standard therapy for acute PE, but newer clot-specific thrombolytic drugs may offer superior benefits with acceptable complication rates in carefully selected patients.  相似文献   

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肺奴卡菌病     
肺奴卡菌病(pulmonary nocardiosis,PN)是由奴卡菌引起的少见而严重的感染,近年来发病率有上升趋势,通常被误诊为其它细菌感染、结核、真菌感染、恶性肿瘤等,有较高的病死率,本文就PN的病原学及流行病学、发病机制、病理变化、临床特点、诊断及鉴别诊断、治疗进展及预后进行综述.  相似文献   

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Sarcoidosis, a granulomatous disorder of unknown etiology, characteristically involves multiple organs. However, pulmonary manifestations typically dominate. Chest radiographs are abnormal in 85 to 95% of patients. Abnormalities in pulmonary function tests are common and may be associated with cough, dyspnea, and exercise limitation. However, one third or more of patients are asymptomatic, with incidental abnormalities on chest radiographs. The clinical course and expression of pulmonary sarcoidosis are variable. Spontaneous remissions occur in nearly two thirds of patients. The course is chronic in up to 30% of patients. Chronic pulmonary sarcoidosis may result in progressive (sometimes life-threatening) loss of lung function. Fatalities ascribed to sarcoidosis occur in 1 to 4% of patients. Although the impact of treatment is controversial, corticosteroids may be highly effective in some patients. Immunosuppressive, cytotoxic, or immunomodulatory agents are reserved for patients failing or experiencing adverse effects from corticosteroids. Lung transplantation is a viable option for patients with life-threatening disease failing medical therapy.  相似文献   

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Pulmonary cachexia   总被引:5,自引:0,他引:5  
Weight loss is a frequent complication in patients with chronic obstructive pulmonary disease (COPD) and is a determining factor of functional capacity, health status, and mortality. Weight loss in COPD is a consequence of increased energy requirements unbalanced by dietary intake. Both metabolic and mechanical inefficiency contribute to the elevated energy expenditure during physical activity, while systemic inflammation is a determinant of hypermetabolism at rest. A disbalance between protein synthesis and protein breakdown may cause a disproportionate depletion of fat-free mass in some patients. Nutritional support is indicated for depleted patients with COPD because it provides not only supportive care, but direct intervention through improvement in respiratory and peripheral skeletal muscle function and in exercise performance. A combination of oral nutritional supplements and exercise or anabolic stimulus appears to be the best treatment approach to obtaining significant functional improvement. Patients responding to this treatment even demonstrated a decreased mortality. Poor response was related to the effects of systemic inflammation on dietary intake and catabolism. The effectiveness of anticatabolic modulation requires further investigation.  相似文献   

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