Cases reported "Pulmonary Fibrosis"

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1/9. 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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2/9. Fibrosing alveolitis in an infant.

    A three month old female infant presented with unproductive cough, diffuse bilateral fine crackles, tachypnoea and failure to thrive despite a four month therapy with beta 2-agonists and antibiotics. A chest radiograph showing bilateral periphilar infiltrates and a patchy infiltrate in the right upper lobe and lingula did not explain the physical examination with diffuse bilateral fine crackles. As the condition did not improve and arterial oxygen tension (PaO2) and oxygen saturation decreased during the following two months, an open lung biopsy was performed. The surgeon described the lungs as rubbery in consistency and histological findings showed patchy mild interstitial fibrosis and thickened alveolar septa. A therapy with prednisone daily was started and given over a period of four months, but did not show sufficient improvement. Only after addition of azathioprine was clinical improvement and normalization of blood gases noted.
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3/9. autopsy confirmation of severe pulmonary interstitial fibrosis secondary to munchausen syndrome presenting as cystic fibrosis.

    Chronic factitious disorder with physical symptoms, or munchausen syndrome, is a well-recognized but uncommonly diagnosed psychiatric condition characterized by the deliberate production of signs and symptoms of disease in order to receive medical attention. Clinical suspicion of this disease is rarely confirmed by autopsy, as the patients usually do not die as a consequence of feigning illness. Here we report the autopsy confirmation of a case of a suspected munchausen syndrome patient who presented with a history of cystic fibrosis. Examination of the lungs demonstrated extensive severe interstitial fibrosis, and polariscopic examination revealed a large quantity of crystalline material throughout the tissue; x-ray diffraction identified the material as talc. Synopses of published cases of munchausen syndrome presenting as cystic fibrosis, and cases of munchausen syndrome with pulmonary talcosis are presented as part of the discussion.
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4/9. Pulmonary fibrosis associated with nabumetone.

    A patient is described who developed a rapid onset of pulmonary fibrosis following treatment with a new non-steroidal anti-inflammatory drug, nabumetone. Resolution of symptoms, physical signs and radiographic changes followed drug withdrawal and steroid therapy.
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5/9. Differential diagnosis of rheumatic disease in the elderly.

    To illustrate the diagnostic problems of rheumatic disease in patients over 60 years of age, 4 cases are presented: 1) osteoarthritis, 2) late-onset rheumatoid arthritis, 3) polymyalgia rheumatica, and 4) pseudogout with crystalline synovitis. In each case the correct diagnosis should have been suspected from the history and physical examination alone, with the laboratory and x-ray findings providing supportive evidence.
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6/9. Desquamative interstitial pneumonia. Computed tomographic findings before and after treatment with corticosteroids.

    Noninvasive methods for diagnosis, assessing prognosis, and following response to treatment in idiopathic pulmonary fibrosis (IPF) have yet to prove their usefulness. We report a patient with desquamative interstitial pneumonia (DIP) in whom computed tomography (CT) suggested the diagnosis by the presence of patchy areas of haziness predominantly in a peripheral distribution. The chest x-ray and physical examinations, and the arterial blood gases showed normal findings after one month of treatment with corticosteroids, at which time the CT scan and lung volumes were still abnormal. At three months, lung volumes and the CT scan were normal. Hazy densities in a peripheral distribution on CT may indicate active IPF associated with a good prognosis.
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7/9. The effects of radiation therapy for Hodgkin's disease in a child with ataxia telangiectasia: a clinical, biological and pathologic study.

    Stage I lymphocyte-predominant Hodgkin's disease was diagnosed in a 44-month-old girl. Although immune deficiency was suspected and iga deficiency demonstrated, the diagnosis of an ataxia-telangiectasia (AT)-like syndrome was not confirmed until eight weeks later when results of studies on the radiosensitivity of cultured skin fibroblasts were available. The child had none of the usual physical stigmata of AT. Severe acute radiation damage followed the treatment of this child with standard doses of radiation therapy. Clinical, pathologic, and radiobiologic correlations are drawn. The diagnosis of a malignant lymphoma disorder in children under the age of five should alert clinicians to the possibility of immune deficiency and, even in the absence of classical physical signs, to AT in particular. Suggestions for the management of future similar cases are put forward.
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8/9. Systemic lupus erythematosus and pulmonary hypertension.

    Pulmonary hypertension is one of the complications in the pulmonary manifestation of SLE. Following a two year SLE history a patient developed pulmonary hypertension although his chest radiograph showed neither pulmonary changes nor signs of hypertension. In two other patients, interstitial fibrosis, pleurisy and pulmonary hypertension were the initial manifestations of SLE. They all complained of dyspnoea and respiratory chest pain. Lung function studies showed restrictive changes, reduced lung compliance and, by two patients, reduced diffusion capacity. Lung biopsy performed in one patient revealed interstitial fibrosis, focal lymphocyte infiltrations and intima proliferation of the arterioles. All three patients were treated with anticoagulants in addition to steroids and cytotoxic drugs. After a period of 8 to 42 months examinations were repeated and all three patients showed improved hypertension and less physical ailment. The radiographs from two patients even revealed an improvement of their pulmonary changes.
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9/9. Interstitial pneumonia in angio-immunoblastic lymphadenopathy with dysproteinemia. A case report with special histopathologic studies.

    A patient presented with characteristic historical, physical, and laboratory findings of angio-immunoblastic lymphadenopathy with dysproteinemia. This newly described entity apparently represents a nonneoplastic proliferation of the B-lymphocyte system with immunoblastic transformation of many lymphocytes and excessive production of immunoglobulins. It is associated with fever, sweats, weight loss, skin rash, lymphadenopathy, splenomegaly, hepatomegaly, and characteristic histologic features of the involved lymph nodes. Noteworthy in the patient reported here are the extent and course of radiographically and clinically evident pulmonary involvement and the biopsy documentation of an interstitial pneumonia marked by histopathologic changes closely resembling those found in the lymph nodes, with immunohistologic demonstration of immunoglobulins in the alveolar walls.
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