Cases reported "Pulmonary Fibrosis"

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1/55. Aluminium dust-induced lung disease in the pyro-powder-producing industry: detection by high-resolution computed tomography.

    OBJECTIVE: The aim of this case study was to investigate the suitability of high-resolution computed tomography (HRCT) for detecting early stages of lung fibrosis induced by aluminium (Al) dust. methods: A 40-year-old worker was studied who had worked as a stamper for 14 years in a plant producing aluminium powder and had been exposed to high levels of aluminium dust during this time. The investigation included the collection of general data on health and details on occupational history, immunological tests, a physical examination, lung function analysis, biological monitoring of Al in plasma and urine, chest x-rays and HRCT. RESULTS: For many years the man has suffered from an exercise-induced shortness of breath. Lung function analysis revealed a reduction of the vital capacity to 57.5% of the predicted value. The Al concentration in plasma was 41.0 micrograms/l (upper reference value 10 micrograms/l) and in urine 407.4 micrograms/l upper reference value 15 micrograms/l, biological tolerance (BAT) value 200 micrograms/l[ at the time of diagnosis. Chest X-ray showed unspecific changes. HRCT findings were characterised by small, centrilobular, nodular opacities and slightly thickened interlobular septae. Exposure to other fibrotic agents could be excluded. CONCLUSIONS: HRCT was more sensitive than chest x-rays for detecting this early stage of Al-dust-induced lung disease. The suitability of HRCT in the surveillance of workers highly exposed to aluminium powder should be evaluated in further studies. Biological monitoring can be used to define workers at high risk.
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keywords = vital capacity, capacity
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2/55. erdheim-chester disease. A case report.

    A 63-year old man had a history of diabetes insipidus, arthralgias and myalgias, weight loss, relapsing fever and malaise. Increased uptake of Tc-99m was found predominantly in distal antebrachia, in distal femurs and in both trochanters and tibias on the bone scintigraphy. The chest radiograph showed reticulonodular pattern and the high resolution computed tomography (HRCT) scans revealed diffuse infiltrative lung disease with small multiple nodules and widening of interlobular septs. Videothoracoscopic lung biopsy and biopsy of tibial lesion were perfomed. The histopathologic examination proved non-Langerhans cell histiocytosis-erdheim-chester disease. Treatment with prednisone reduced the pain and fever and improved the vital capacity of the lungs while the changes in the lungs and bones remained unchanged.
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keywords = vital capacity, capacity
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3/55. Interstitial lung disease resistant to corticosteroid therapy. Report of three cases treated with azathioprine or cyclophosphamide.

    Three patients with severe progressive interstitial lung disease refractory to steroid therapy were treated with immunosuppressive drugs. biopsy material of one showed mainly fibrosis, while that of the second showed interstitial pneumonitis as well; both patients received azathioprine. cyclophosphamide was employed in the third patient with systemic vasculitis and massive hemoptysis. All patients had reduced lung volumes and abnormal gas exchange, which continued to worsen on high doses of steroids. In patients 1 and 2, there was long-term stabilization of lung function, while pulmonary physiologic abnormalities in the patient with vasculitis reverted to normal on five months of cyclophosphamide. Although the etiology of most forms of interstitial lung disease is unknown, several reports suggest at least a partial immunologic basis. Abatement in progression of disease in this small series would suggest that a trial of immunosuppressive drugs be considered in interstitial lung disease when steroid therapy fails.
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keywords = volume
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4/55. Withdrawal of life support: intensive caring at the end of life.

    The technology and expertise of critical care practice support patients through life-threatening illnesses. Most recover; some die quickly; others, however, linger--neither improving nor acutely dying, alive but with a dwindling capacity to recover from their injury or illness. Management of these patients is often dominated by the question: Is it appropriate to continue life-sustaining therapy? patients rarely participate in these pivotal discussions because they are either too sick or too heavily sedated. As a result, the decision often falls to the family or the surrogate decision maker, in consultation with the medical team. Decisions of such import are emotionally stressful and are often a source of disagreement. Failure to resolve such disagreements may create conflict that compromises patient care, engenders guilt among family members, and creates dissatisfaction for health care professionals. However, the potential for strained communications is mitigated if clinicians provide timely clinical and prognostic information and support the patient and family with aggressive symptom control, a comfortable setting, and continuous psychosocial support. Effective communication includes sharing the burden of decision making with family members. This shift from individual responsibility to patient-focused consensus often permits the family to understand, perhaps reluctantly and with great sadness, that intensive caring may involve letting go of life-sustaining interventions.
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5/55. cyclophosphamide-induced late-onset lung disease.

    We report the case of a 58-year-old woman who was considered to have died of cyclophosphamide (CPA)-induced late-onset lung disease. She underwent a right-sided mastectomy due to breast cancer at the age of 50 followed by daily administration of 50 mg CPA and 20 mg tamoxifen for 2 years. A refractory cough and dyspnea began at the age of 56. Chest radiographs revealed diffuse infiltrates and pleural thickening. Her vital capacity was markedly reduced. Corticosteroid therapy was ineffective. The disease rapidly progressed with occasional episodes of pneumothoraces to her death. Postmortem examination revealed pulmonary fibrosis with marked elastosis.
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keywords = vital capacity, capacity
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6/55. Acute respiratory failure after interferon-gamma therapy of end-stage pulmonary fibrosis.

    Interferon (IFN)-gamma was recently proposed as a treatment for idiopathic pulmonary fibrosis. We report on four patients who developed acute respiratory failure with new alveolar opacities after 2 (two patients), 6, and 35 injections of IFN-gamma-1b. All four patients had advanced idiopathic pulmonary fibrosis (total lung capacity less than 45% predicted or carbon monoxide diffusion capacity less than 30% predicted), and two patients had familial pulmonary fibrosis. No other cause of deterioration was found. Refractory hypoxemia led to death in three cases and to lung transplantation in one case. Pathologic studies in two patients showed diffuse alveolar damage lesions with preexisting usual interstitial pneumonia. These cases suggest that IFN-gamma therapy can induce an acute respiratory failure in patients with end-stage idiopathic pulmonary fibrosis.
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ranking = 0.040437458345738
keywords = capacity
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7/55. bronchiectasis in children with lymphocytic interstitial pneumonia and acquired immune deficiency syndrome. Plain film and CT observations.

    In a review of 77 hiv positive children seen between 1981 and 1990, 32 were diagnosed as having lymphocytic interstitial pneumonitis). Four of the lip group developed bronchiectasis, a finding not previously reported. The precise factors leading to the bronchiectasis are unclear. All patients had chronically consolidated lung with volume loss. A history of recurrent bacterial superinfection was not noted in any of the cases. With more cases of hiv positive children living longer, bronchiectasis, long known to occur in primary immunologic disorders, will probably be more frequently noted.
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ranking = 2.700212638891E-5
keywords = volume
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8/55. bronchiolitis obliterans organizing pneumonia and scleroderma.

    Pulmonary involvement in scleroderma is characterized by interstitial fibrosis and pulmonary hypertension. Although bronchiolitis obliterans organizing pneumonia (BOOP) may be associated with a variety of connective tissue diseases and their treatment, there are only rare reports of bronchiolitis associated with scleroderma. We describe 2 patients with scleroderma and rapidly evolving pulmonary infiltrates, which upon biopsy showed histologic findings of BOOP. Each patient had severe restrictive lung disease and markedly diminished diffusion capacity. Treatment with high dose prednisone showed a good response in one patient. The rapid course of pulmonary findings in these patients differs from the usual course of pulmonary fibrosis in scleroderma. Although BOOP is a rare finding in scleroderma, our findings suggest that rapid pulmonary decompensation or atypical findings for pulmonary fibrosis should prompt consideration for an open lung biopsy. Finding a potentially steroid responsive disorder in an otherwise steroid unresponsive disorder has important clinical implications.
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9/55. Combined nitrofurantoin toxicity to liver and lung.

    nitrofurantoin is an antibiotic commonly used for prophylaxis and treatment of urinary tract infections. Pulmonary and hepatic toxicity are rare side effects of this agent. The simultaneous occurrence of pulmonary fibrosis and chronic active hepatitis in a patient undergoing long-term nitrofurantoin therapy is reported. The presence of pulmonary toxicity was evidenced by infiltrates on chest radiographs and impaired diffusion capacity during pulmonary function tests. Prolonged elevation of liver enzyme concentrations together with the presence of increased antibody titers (anti-smooth muscle antibody, antinuclear antibody) was suggestive of chronic hepatitis, a diagnosis corroborated by liver biopsy findings. After discontinuation of nitrofurantoin therapy, the patient had a full recovery. The infiltrates initially found on chest radiographs disappeared, and laboratory parameters normalized without the need for corticosteroid therapy.
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10/55. Hard metal interstitial pulmonary disease associated with a form of welding in a metal parts coating plant.

    We describe two cases of hard metal pulmonary disease (one fatal) in workers employed in the same area of a metal coating plant using the detonation gun process for applying a durable metal surface to metal parts. In this form of welding, a mixture of powdered metals, including tungsten carbide and cobalt, is heated by ignition of a flammable gas and propelled from the end of the "gun" at high temperature and velocity to form a welded metal coating. This process is done in an enclosed chamber and with each application, large volumes of fine aerosols are created. inhalation exposure to hard metal may occur during the mounting and removal of the metal parts between applications, in spite of engineering controls and industrial hygiene surveillance. One of the cases presented with minimal chest x-ray abnormalities and an obstructive pattern on pulmonary function testing, although subsequent open lung biopsy showed diffuse interstitial pulmonary fibrosis. The fact that two cases of hard metal pulmonary interstitial disease occurred where thorough exposure control procedures and a surveillance program for cobalt were in place may indicate the need for revisions of the current technology used when hard metal is applied in the detonation gun process.
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keywords = volume
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