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1/106. Acute respiratory distress syndrome in a welder exposed to metal fumes.

    A 43-year-old man began having malaise, chills, and fever 12 hours after cutting a galvanized steel grating with an acetylene torch at work. Over the next 72 hours, his symptoms persisted and became worse with progressive shortness of breath. He was admitted to the hospital and begun on antibiotics and steroids. The next day his condition had deteriorated to the point that he had to be intubated. Chest x-ray film and computed tomography showed patchy and interstitial infiltration bilaterally, consistent with acute respiratory distress syndrome. Open lung biopsy showed focal mild interstitial pneumonia. Multiple laboratory studies were negative for an infectious or an immune process. The patient remained on mechanical ventilation for 10 days and was discharged from the hospital 2 days after extubation. He continued to improve, with minimal symptoms and a return to normal activity levels several months after the incident with no continued treatment. Re-creation of his exposure was done under controlled circumstances, with air sampling revealing elevated air levels for cadmium and zinc and borderline levels of arsenic, manganese, lead, and iron.
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2/106. Nylon flock-associated interstitial lung disease.

    A work-related interstitial lung disease has been diagnosed in workers at five nylon flock facilities in three different states and a Canadian province. The National Institute for Occupational safety and health hosted a workshop at which consulting pulmonary pathologists reviewed lung tissue samples from all the cases for which lung biopsy material was available (15 of 20 cases known in January 1998). After independent review and scoring of these lung tissue specimens, the pathologists reached consensus that the histopathological findings revealed a characteristic lesion-a lymphocytic bronchiolitis and peribronchiolitis with lymphoid hyperplasia represented by lymphoid aggregates. The pathologists noted that the pathological findings were distinctive when compared with known lung conditions. The clinical presentation for the cases generally included cough, dyspnea, restrictive ventilatory defect with reduction in diffusing capacity, and interstitial markings on chest radiographs or high-resolution computed tomography (HRCT) scans. Six of the cases improved after removal from workplace exposure without medical treatment. Six others, who had recovered with medical treatment and removal from the workplace, had relapses in both symptoms and objective findings after attempting to return to nylon flock work. With this and other evidence supporting the existence of chronic interstitial pneumonitis associated with nylon flock processing, workshop participants recommended surveillance for early identification of affected workers and their removal from further workplace exposure.
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3/106. pulmonary veno-occlusive disease, antiphospholipid antibody and pulmonary hypertension in an adolescent.

    pulmonary veno-occlusive disease (PVOD) is a rare cause of pulmonary hypertension (PH); Antiphospholipid antibody (APL) is another known cause of pulmonary hypertension, due to recurrent pulmonary thromboembolism. The coincidence of both causes, PVOD and APL, without thromboembolism, in PH has not been reported previously in children. A 12.5-year-old boy presented with a one year history of fatigue. Pulmonary hypertension was diagnosed by echocardiography. Pulmonary function tests revealed a moderate restrictive pattern and elevated granulocytes were detected in bronchoalveolar lavage. An isolated high-titer APL was detected. Open lung biopsy established the diagnosis of PVOD, with no evidence of pulmonary thrombosis, but with accompanying interstitial and alveolar cellular infiltration. We speculate that APL may have played a role in the pathogenesis of PVOD. prednisone improved the symptoms of the interstitial pneumonitis and was stopped; on follow up of 30 months, the patient ist in stable condition on therapy with nifedipin, phenprocoumon and digoxin. CONCLUSIONS: PVOD and APL may be present simultaneously as a rare cause of PH. Interstitial pneumonitis may accompany PVOD and produce the leading symptoms. Open lung biopsy is essential for early establishment of the diagnosis.
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ranking = 7362.5543425606
keywords = alveolar
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4/106. Diffuse pulmonary infiltrates in immunocompromised patients.

    The differential diagnosis of bilateral interstitial pulmonary infiltrates in immunocompromised patients is very extensive. We describe two immunocompromised patients with diffuse pulmonary infiltrative changes. Bronchoscopic bronchoalveolar lavage after orotracheal intubation using topical anaesthesia combined with mild sedation in an ICU setting is safe in critically ill patients and often yields a conclusive diagnosis.
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ranking = 3681.2771712803
keywords = alveolar
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5/106. Infantile pulmonary alveolar proteinosis with interstitial pneumonia: bilateral simultaneous lung lavage utilizing extracorporeal membrane oxygenation and steroid therapy.

    An infant with refractory pulmonary alveolar proteinosis (PAP) associated with severe interstitial pneumonia is described. Although she was treated by bilateral simultaneous lung lavage utilizing extracorporeal membrane oxygenation and steroid therapy, she died of progressive respiratory failure 28 days after admission. Histologic examination of lung autopsy specimen showed only partial alveolar spaces to be filled with a dense PAS positive granular eosinophilic material and showed severe interstitial pneumonia with marked fibrosis of alveolar walls and interstitium. The lung lavage seemed to be effective for PAP because the effluent fluid sufficiently became clear and the PAS positive material was detected only in partial alveoli. The full venoarterial cardiopulmonary bypass with extracorporeal membrane oxygenation seemed to be very useful to support bilateral lung lavage for small infants. The refractory symptoms and failure of treatment were resulted from the association of severe interstitial pneumonia. In neonates or infants with PAP and severe interstitial pneumonia with poor response for steroid therapy, the lung transplantation should be considered.
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ranking = 25768.940198962
keywords = alveolar
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6/106. giant cell arteritis can be associated with T4-lymphocytic alveolitis.

    We describe three patients with histologically proven giant cell arteritis who presented with respiratory complaints. In one patient, dry cough and dyspnoea dominated the clinical picture. In the other two patients, a diagnosis of giant cell arteritis was readily suspected by the presence of typical complaints, although both patients spontaneously mentioned a persistent cough and dyspnoea, respectively. Radiographs of the chest were normal. Lung function tests, including a carbon monoxide (CO)-diffusion capacity measurement, were always normal. Broncho-alveolar lavage fluid examination showed a normal cell count but an increased number of lymphocytes (16-61%) with a predominance of T4-lymphocytes (65.5-84.5%). We conclude that respiratory complaints and T4-lymphocytic alveolitis can be associated with giant cell arteritis.
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keywords = alveolar
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7/106. Low dose cabergoline induced interstitial pneumonitis.

    Certain dopaminergic anti-Parkinson drugs (ergolines) have repeatedly been identified as a cause of pleuropulmonary disease with a focus on serosal cell damage. Recently, a pathogenetic link between ergolines and prior asbestos exposure was suggested, as regards the development of pleural pathology. This report describes a patient with idiopathic Parkinson's disease, who was on a multiple drug regimen including low dose cabergoline. The patient developed a febrile illness with widespread bilateral lung infiltrations nonresponsive to beta-lactam and macrolide antibiotics. bronchoalveolar lavage and transbronchial lung biopsy showed a "hypersensitivity-like" interstitial lung disease, which cleared almost completely within 2 months after simple drug withdrawal. Circumstantial evidence suggests a so far undescribed adverse lung reaction to cabergoline, devoid of the more usual pleural changes.
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ranking = 3681.2771712803
keywords = alveolar
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8/106. Deficiency of lamellar bodies in alveolar type II cells associated with fatal respiratory disease in a full-term infant.

    We report a case of a full-term female infant who presented with severe respiratory distress shortly after birth and died at 23 d of age with unremitting respiratory failure. Infectious and other known causes of respiratory disease in this clinical setting were excluded. Examination of a lung biopsy showed abnormal lung parenchyma with features reminiscent of desquamative interstitial pneumonitis. Ultrastructural studies revealed that alveolar type II cells lacked cytoplasmic lamellar bodies, while other organelles appeared normal. Histochemical and immunohistochemical investigations indicated normal alveolar type II cell marker expression including surfactant proteins (SP-A, SP-B, pro-SP-B, and pro-SP-C). Mutations in the coding sequences of the SP-B gene were excluded as a cause of disease. This case appears to be a novel congenital defect affecting the pulmonary surfactant system. The cellular abnormality may involve the assembly of cytoplasmic lamellar bodies in alveolar type II cells-the principal storage site of pulmonary surfactant.
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ranking = 25768.940198962
keywords = alveolar
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9/106. A diffuse alveolar hemorrhage in a human T-lymphotropic virus type I carrier with acute cerebellar ataxia and interstitial pneumonitis: an autopsy case report.

    A 76-year-old HTLV-I-positive male with acute cerebellar ataxia was suffering from dyspnea on exertion. Chest CT suggested interstitial pneumonitis. methylprednisolone pulse therapy improved his symptoms and chest CT findings. Twelve months after discharge, when the prednisolone dose was tapered to 5 mg every other day, his lung lesion recurred. The lesion responded initially to steroid therapy. However, hypoxemia intractable to steroid pulse therapy developed and the patient died of respiratory failure. The autopsy revealed diffuse alveolar hemorrhage with no finding of vasculitis. This is the first case report of diffuse alveolar hemorrhage in an HTLV-I carrier.
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ranking = 22087.663027682
keywords = alveolar
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10/106. Pulmonary lymphangitic sarcomatosis from cutaneous angiosarcoma: an unusual presentation of diffuse interstitial lung disease.

    Pulmonary lymphangitic sarcomatosis (PLS) is not much recognized clinically although it shows similar pathological patterns and diagnostic features to pulmonary lymphangitic carcinomatosis (PLC). We report a case with hand angiosarcoma whose chest X-ray findings revealed a diffuse interstitial pattern consistent with lymphangitic spreading. The final diagnosis was made by open lung biopsy. The clinical, diagnostic and pathological features of this disease process are reviewed.
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