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1/15. Rapidly progressive antineutrophil cytoplasm antibodies associated with pulmonary-renal syndrome in a 10-year-old girl.

    CONTEXT: The term pulmonary-renal syndrome has been used frequently to describe the clinical manifestations of a great number of diseases in which pulmonary hemorrhage and glomerulonephritis coexist. The classic example of this type of vasculitis is Goodpastures syndrome, a term used to describe the association of pulmonary hemorrhage, glomerulonephritis and the presence of circulating antiglomerular basement membrane antibodies (anti-GBM). Among the several types of systemic vasculitides that can present clinical manifestations of the pulmonary-renal syndrome, we focus the discussion on two types more frequently associated with antineutrophil cytoplasm antibodies (ANCA), microscopic polyangiitis and Wegener's granulomatosis, concerning a 10 year old girl with clinical signs and symptoms of pulmonary-renal syndrome, with positive ANCA and rapidly progressive evolution. CASE REPORT: We describe the case of a 10-year-old girl referred to our hospital for evaluation of profound anemia detected in a primary health center. Five days before entry she had experienced malaise, pallor and began to cough up blood-tinged sputum that was at first attributed to dental bleeding. She was admitted to the infirmary with hemoglobin = 4 mg/dL, hematocrit = 14 %, platelets = 260,000, white blood cells = 8300, 74 % segmented, 4 % eosinophils, 19 % lymphocytes and 3 % monocytes. Radiographs of the chest revealed bilateral diffuse interstitial alveolar infiltrates. There was progressive worsening of cough and respiratory distress during the admission day, when she began to cough up large quantities of blood and hematuria was noted. There was rapid and progressive loss of renal function and massive lung hemorrhage. The antineutrophil cytoplasm antibody (ANCA) test with antigen specificity for myeloperoxidase (anti-MPO) was positive and the circulating anti-GBM showed an indeterminate result.
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2/15. Pro-gastrin-releasing peptide (ProGRP), neuron specific enolase (NSE), carcinoembryonic antigen (CEA) and cytokeratin 19-fragments (CYFRA 21-1) in patients with lung cancer in comparison to other lung diseases.

    The study presents data comparing the new tumour marker, ProGRP, with the established markers, NSE, CYFRA 21-1 and CEA in the diagnosis of lung cancer. ProGRP as well as NSE have been reported to be useful markers for staging and monitoring treatment in patients with small cell lung cancer (SCLC). In order to determine the differences in the sensitivity and/or specificity particularly with regard to benign lung diseases, the present study investigated ProGRP in comparison to NSE, CEA and CYFRA 21-1 usually used in lung cancer. ProGRP was quantitatively detectable with an ELISA. So far 192 newly-diagnosed lung cancer patients including 51 SCLC have been examined. Served as controls: 124 subjects i.e. 50 patients with pneumoconiosis, 22 patients with obstructive airway diseases, 34 patients with acute inflammatory lung diseases and 18 healthy persons. Significantly elevated tumour marker concentrations were found for ProGRP and NSE in SCLC. At a specificity of 95%, ProGRP and NSE showed comparable sensitivities (68.6% and 74.5%) in SCLC. ProGRP also reached high levels in patients with limited disease status (sensitivity ProGRP: 72.2%, NSE 66.7%). Initial follow-up studies indicated that ProGRP can be used to monitor disease under chemotherapy. In non-small cell lung carcinomas, CYFRA 21-1 was the leading marker with 58.9% sensitivity where ProGRP seldom revealed positive results. ProGRP is a valuable tumour marker for the detection and monitoring of SCLC and a good tool for discriminating NSCLC versus SCLC.
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3/15. Transfusion-related acute lung injury resulting from designated blood transfusion between mother and child: a report of two cases.

    Transfusion-related acute lung injury (TRALI) is an underdiagnosed serious complication of blood transfusion characterized by the rapid onset of respiratory distress, hypoxia, and noncardiogenic pulmonary edema during or soon after blood transfusion. The presence of anti-HLA and/or antigranulocyte antibodies in the plasma of donors is implicated in the pathogenesis of TRALI. We report 2 cases of TRALI that were caused by designated blood transfusion between mothers and their daughters; one in a 4-month-old girl who received designated packed RBCs donated by her mother and the second in a 78-year-old mother who received blood from her daughter. In both cases, examination of mother's serum revealed panel-reactive cytotoxic HLA antibodies. It is most likely that the mothers were sensitized from earlier pregnancy and produced HLA antibodies against the daughters' paternally derived hla antigens. Designated blood transfusion between multiparous mothers and children might add an additional transfusion-related risk owing to the higher likelihood of the HLA antibody-antigen specificity between mother and child.
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4/15. amiodarone-induced pulmonary toxicity. Immunoallergologic tests and bronchoalveolar lavage phospholipid content.

    amiodarone (A) is a widely-used antiarrhythmic drug. Pulmonary toxicity is the most serious adverse effect with an estimated mortality of 1 to 33 percent. In order to determine an element helpful for diagnosis, we examined four patients with amiodarone-induced pulmonary toxicity, three patients treated with A, without evidence of pulmonary toxicity but with a main underlying pulmonary disease, and four healthy volunteers. Daily and cumulative doses or duration of treatment were similar in the first two groups. Pulmonary function tests (spirometry, CO-diffusing capacity, arterial blood gases), roentgenographic examinations, pulmonary biopsies or immunoallergologic tests (skin reaction, lymphoblastic transformation test and human basophile degranulation test) did not provide any discriminatory element. In APT , we observed an increased cellularity of the bronchoalveolar lavage. Neither the differential cell count nor the presence of foamy macrophages were distinguishable between APT and APT-. The phospholipid composition of BAL fluid showed a decreased total phospholipid and phospholipid/protein ratio in all patients compared to normal subjects. These changes reflect more the severity of pulmonary disease than the specificity of the causative agent. However, we observed that the unique PL which decreases in APT- and remains normal in APT is phosphatidyl-serine phosphatidylinositol (PS PI). This has to be confirmed and should be evaluated at different stages of the disease to determine an eventual specific element. We conclude that there are no data currently available to establish the diagnosis of APT except perhaps for the analysis of BAL PL content.
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ranking = 1
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5/15. Pulmonary plasma-cell granuloma.

    A large pulmonary plasma-cell granuloma (PCG) mimicking a mediastinal germinal-cell tumor in a 9-year-old boy is presented. The nonspecificity of the tumoral calcifications and of the radiographic and CT findings in pulmonary PCG is demonstrated.
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ranking = 1
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6/15. Diagnostic value of lung uptake of indium-111 oxine-labeled white blood cells.

    One hundred sixty-two white-blood-cell scans were retrospectively reviewed to determine the sensitivity and specificity of the test for pulmonary and pleural infection. All scans were performed 18-24 hr after injection of indium-111 oxine-labeled autologous or donor cells. Pulmonary activity was graded on a scale of 0-4: 0 = equal to soft tissue; 1 = greater than soft tissue but less than rib; 2 = equal or greater than rib but less than liver; 3 = equal or greater than liver but less than spleen; 4 = equal to spleen. Activity was also characterized as being focal or diffuse. The white-blood-cell scan findings were correlated with the clinical diagnosis on the basis of physical examination, laboratory results, chest radiographs, clinical course, and pathologic studies when available. As pulmonary activity increased from grade 1 to 4, sensitivity declined from 93% to 14% and specificity increased from 64% to 100%. The sensitivity and specificity of focal uptake were 31% and 89% vs 62% and 74% for diffuse pulmonary activity. Making a distinction between focal and diffuse activity did not improve the specificity of low grades of pulmonary activity. The white-blood-cell scan can be very sensitive or very specific for pulmonary or pleural infection, depending on the criteria selected for a positive scan.
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ranking = 4
keywords = specificity
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7/15. Fluid within preexisting pulmonary air-spaces: a potential pitfall in the CT differentiation of pleural from parenchymal disease.

    There have been many reports of the ability of CT to distinguish between parenchymal and pleural disease. The purpose of this report is to describe the appearance of seven cases of intraparenchymal fluid-filled air-spaces (bullae or lung cysts) in which the CT findings may resemble those of pleural disease and, thus, cast doubt on the specificity of the established criteria.
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ranking = 1
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8/15. Variable CT appearance of plasma cell granuloma of the lung.

    plasma cell granuloma of the lung is an uncommon benign inflammatory pseudotumor. Although it typically presents as a solitary mass on chest radiography, several chest film appearances have been described. Three cases are presented that illustrate the variable range and nonspecificity of CT appearances of plasma cell granuloma of the lung. Computed tomography is useful in the evaluation of these lesions primarily to establish important anatomic relationships associated with each of the various manifestations.
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ranking = 1
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9/15. Scintigraphic detection of pulmonary hemorrhage using Tc-99m-sulfur colloid.

    Pulmonary hemorrhage, whether in the form of hemoptysis or bleeding into the pleural space, may be a life threatening problem. While fiberoptic bronchoscopy and selective bronchial and intercostal angiography are the major diagnostic modalities, these techniques are not without risks. Two patients are described-one with massive hemoptysis from a bronchogenic carcinoma; another with pleural bleeding from a torn intercostal artery-in whom Tc-99m-sulfur colloid scintigraphy accurately located the source of hemorrhage. We conclude that Tc-99m-sulfur colloid scintigraphy may be a helpful noninvasive method to increase the sensitivity and specificity of fiberoptic bronchoscopy and bronchial angiography in patients with massive pulmonary bleeding.
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10/15. color Doppler ultrasound signals of thoracic lesions. Correlation with resected histologic specimens.

    Sixty-eight patients with thoracic lesions (48 with lung cancer and 20 with benign lesions) underwent color Doppler ultrasound (US) examinations. Of those, 21 patients (13 with lung cancer and eight with benign lesions) also received resections, and the correlation between color Doppler US signals and resected histologic specimens was evaluated. Our results showed that three patterns of color Doppler US signals could be detected and confirmed: pulsatile flow (artery), constant flow, and triphasic flow (pulmonary vein). Among the 48 patients with lung cancer, pulsatile flow, constant flow, and/or triphasic flow were detected in 34 (71%), 24 (50%), and 14 (29%), respectively. Among the 20 patients with benign lesions, only pulsatile flow and/or triphasic flow were detected in nine (45%) and eight (40%), respectively. From the correlation between color Doppler US signals and histologic specimens, constant flow was representative of the true neovascularity of lung cancers, and it was valuable for differentiating lung cancers from benign lesions (p = 0.00008, sensitivity = 0.50, and specificity = 1.0). Although color Doppler US still had some limitations in detecting blood vessels in thoracic lesions, the correlation between the vascularity represented by color Doppler US signals and histologic specimens was excellent. We conclude that color Doppler US is a valuable method for assessing blood flow in thoracic lesions and differentiating lung cancers from intrapulmonary benign lesions.
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ranking = 1
keywords = specificity
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