Cases reported "Lung Diseases"

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1/50. Immunohistochemical study of a patient with diffuse pulmonary corpora amylacea detected by open lung biopsy.

    The chest radiographs of an asymptomatic 58-year-old Japanese man with pulmonary corpora amylacea (PCA) revealed bilateral patchy and nodular infiltrates. Lung specimens obtained by open lung biopsy were histochemically and immunohistochemically analyzed. In all sections of dissected lung tissue, hematoxylin and eosin staining revealed homogeneous eosinophilic acellular round bodies (50-100 microm in diameter) containing granular black pigments in the alveolar spaces. Some round bodies were surrounded and phagocytized by alveolar macrophages. The laminated round bodies stained positively with PAS and congo red. In addition, many of the rounded bodies contained particles which stained positively with berlin blue. Immunohistochemical staining for epithelial membrane antigen (EMA) as well as PE-10 was distinctively positive. This is a very rare case of diffuse PCA found by open lung biopsy. Immunohistochemical examination suggested that PCA consisted of pulmonary surfactant protein and epithelial membrane antigen.
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2/50. gastric outlet obstruction and pulmonary infiltrate in a patient with Crohn's disease: successful treatment by Billroth-II-resection.

    We present a 28-year-old women with a 3 yr history of duodenal ulcers. Following four treatment attempts to eradicate helicobacter pylori she was admitted because of gastric outlet obstruction and a weight loss of 20 kg within the last two years. endoscopy and x-ray showed a circular inflammatory stenosis of the proximal duodenum extending over 8 cm. Additionally, chest x-ray showed a circumscript infiltrate in the third segment of the right lung. Mycobacterial infection could be excluded. Ileocolonoscopy and small intestinal follow-through beyond the duodenum were unremarkable, and Zollinger-Ellison-syndrome was ruled out. Bronchopulmonary histology showed intramucosal epithelioid-cell granulomas and bronchiolitis obliterans. Because the patient did not improve under conservative therapy a Billroth-II-resection was carried out. Histologically the resected specimen showed Crohn-like lesions. Postoperatively, severe peripheral arthritis was treated by steroids over 6 weeks. At follow-up the patient regained 20 kg and was free of symptoms without any medication. The pulmonary infiltrate had subsided almost completely. In summary, this extremely rare coincidence of isolated stenosing duodenal Crohn's disease and pulmonary involvement was successfully treated by Billroth-II-resection. This course of disease is compatible with the hypothesis that Crohn's disease may be maintained by antigens derived from ingested food.
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3/50. 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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4/50. hepatitis b-related polyarteritis nodosa complicated by pulmonary hemorrhage.

    A 34-year-old man presented with fever, weight loss, paresthesia, abdominal pain, and hypertension. He had hepatitis b antigenemia, with negative antineutrophil cytoplasmic antibody, antinuclear antibody, and antiglomerular basement membrane serology results. Renal arteriography showed multiple intrarenal microaneurysms. In spite of therapy with antiviral agents (lamivudine, famciclovir), prednisone, cyclophosphamide, and plasmapheresis, renal function deteriorated. He later developed rapidly progressive dyspnea and hemoptysis. Diffuse alveolar hemorrhage was confirmed by bronchoscopy. He died of respiratory failure. The cause of pulmonary hemorrhage in this case of polyarteritis nodosa is unclear, but may include underlying capillaritis, cocaine-induced pulmonary hemorrhage, or recurrent attacks of malignant hypertension.
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5/50. Clinicopathological and immunohistochemical features of the severe pulmonary form of leptospirosis.

    Four cases of severe pulmonary form of leptospirosis (SPFL) are described. In all four of these blood culture proven cases, there was severe pulmonary injury characterized by alveolar hemorrhage and acute respiratory failure. Three patients died in less than 48 hours after onset of the first respiratory signs. Leptospiral antigen detection in lung tissues was positive by immunoperoxidase in all three of these cases, suggesting that the microorganism exerts a local direct destructive action. patients with SPFL should be carefully monitored, as the abrupt onset of severe alveolar hemorrhage can lead to respiratory insufficiency and death. The authors emphasize the importance of radiological findings and blood gas analysis for prompt clinical diagnosis, and suggest that corticosteroids, associated with antibiotics, early respiratory support, and platelet transfusions are useful as an attempt to prevent further development of SPFL.
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6/50. Respiratory failure due to micronodular type II pneumocyte hyperplasia.

    AIMS: To report the first case of respiratory failure due to micronodular type II pneumocyte hyperplasia. methods AND RESULTS: biopsy, explant and autopsy material from a 16-year-old girl, a smoker, with no personal or family history of tuberous sclerosis, who died following lung transplantation necessitated by progressive respiratory failure, was evaluated histologically. Micronodular pneumocyte hyperplasia was identified histologically as the cause of the respiratory failure. Foci of hyperplastic type II pneumocytes measuring up to 4 mm were widely scattered through the lungs. The hyperplastic cells had abundant eosinophilic cytoplasm, vesicular nuclei and prominent nucleoli. immunohistochemistry showed that they stained for cytokeratins AE1, AE3 and CAM 5.2 and for epithelial membrane antigen (EMA), but not for carcinoembryonic antigen (CEA), S100, smooth muscle actin, CD68 and HMB-45. CONCLUSIONS: Although micronodular type II pneumocyte hyperplasia is usually of no clinical significance, in our patient the process was so florid as to cause respiratory failure, which was severe enough to necessitate lung transplantation.
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7/50. TRALI due to granulocyte-agglutinating human neutrophil antigen-3a (5b) alloantibodies in donor plasma: a report of 2 fatalities.

    BACKGROUND: TRALI is usually an immunologic reaction to WBC antibodies in infused plasma and ranks second only to ABO mismatch as a cause of transfusion-associated death. Implicated donors are usually multiparous women (>/=3 pregnancies). STUDY DESIGN AND methods: Two fatal cases of TRALI were evaluated by reviewing clinical and laboratory findings and characterizing alloantibodies present in donor plasma. Investigation for WBC antibodies was by lymphocytotoxicity (LCT), FlowPRA (FlowPRA, One Lambda, Inc.) and granulocyte immunofluorescence and agglutination assays. Patient 1 was a 62-year-old man with chronic T-cell lymphocytic leukemia, and Patient 2 was a 54-year-old woman undergoing a cadaveric kidney transplant. Both patients developed acute respiratory distress and hypotension during (Patient 1) and approximately 30 minutes after (Patient 2) transfusion. Fulminant pulmonary edema ensued in both cases necessitating mechanical ventilation and both patients died within 24 hours of the onset of respiratory complications. RESULTS: The donors of the implicated blood components were women with a history of two pregnancies but no blood transfusions. Weak apparently panreactive granulocyte antibodies were detected with flow cytometry. However, in the granulocyte agglutination test, strong antibodies specific for human neutrophil antigen (HNA)-3a (5b) were identified in both donors. CONCLUSION: It is concluded that female blood donors with only two previous pregnancies can form clinically important granulocyte-reactive alloantibodies leading to fatal TRALI reactions in recipients. The sometimes devastating consequences of TRALI should prompt the development of strategies to prevent or reduce its incidence. Further research is warranted to investigate recipient and donor factors responsible for TRALI, including whether 5b (HNA-3a) alloantibodies are especially prone to cause severe reactions, and to better characterize the HNA-3a (5b) antigen, particularly at the molecular level.
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8/50. Systemic nocardiosis following allogeneic bone marrow transplantation.

    Five cases of systemic nocardia infection were diagnosed among 301 allogeneic bone marrow transplant recipients. A sixth case included in this report received her transplant at another institution. The cumulative annual incidence rate of this infection was 1.75%. All patients had been treated previously for acute graft-versus-host disease (GVHD). At the time of diagnosis of systemic nocardia infection, a median of 198 (range 148-1121) days after transplantation, all patients had extensive chronic GVHD and were taking 2 to 3 immunosuppressive medications. Prior to diagnosis of nocardia infection patients had experienced multiple opportunistic infections, including infections with mycobacterium avium-intracellulare, pneumocystis carinii, and cytomegalovirus antigenemia. Treatment with trimethoprim-sulfamethoxazole (TMP-SMX), ceftriaxone, or carbapenem antibiotics resulted in a median survival of 219 days from the time of diagnosis and an actuarial 1-year survival of 40%. All patients who received more than 2 weeks of therapy were cured of their infections. Notably, 5/6 patients in this cohort were unable to take TMP-SMX because of myelosuppression. In comparison with randomly selected control patients, the use of pentamidine for prevention of P. carinii infection was associated with a marginal increase in the risk of nocardia infection. We postulate that the use of TMP-SMX may be of benefit in the prophylaxis of infections other than P. carinii in patients with chronic GVHD.
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9/50. 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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10/50. Single hospital experience of TRALI.

    BACKGROUND: TRALI is a serious adverse effect of blood transfusion. There is evidence that the condition is underrecognized and underreported. STUDY DESIGN AND methods: This study was an observational study carried out in a single hospital. RESULTS: Eleven cases of TRALI were recognized over 12 years. In 10 cases the implicated donor unit was FFP and in 1 case uncertain. All implicated donors were parous women. In 4 cases the presumed causative antibodies were to an HLA class II antigen only. Specific anti-neutrophil antibodies, possibly causative, were detected in 1 case only. Ten of the 11 cases required mechanical ventilatory support. Five persons died as a result of the TRALI. The observed incidence of TRALI caused by FFP is 1 in 7900 units transfused. CONCLUSION: TRALI is the most common serious adverse effect of blood transfusion in our hospital. antibodies to HLA class II antigens should be looked for routinely when investigating a possible case of TRALI.
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