Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0034069 (pulmonary fibrosis)
7,050 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 50-year-old man with pulmonary fibrosis and COPD presented with worsening cough, dyspnea, chest pain, and hypoxemia of no readily apparent etiology, approximately four weeks after insertion of a transtracheal oxygen therapy catheter. Despite vigorous bronchial hygiene therapy, the patient died. Autopsy revealed obstruction of the trachea by a large mucous ball. We point out the nonspecificity of physical and radiologic findings associated with this condition and suggest that serial flow-volume loop analysis or earlier use of fiberoptic bronchoscopy might have been of assistance in premortem diagnosis of the mucous plug.
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PMID:Fatal airway obstruction caused by a mucous ball from a transtracheal oxygen catheter. 160 Aug 12

Despite suspicion that inhalation of zirconium should be capable of causing human pulmonary disease, documentation of zirconium pneumoconiosis in humans has been lacking. We studied a likely case of zirconium compound-induced pulmonary fibrosis. The diagnosis was based on the following: (1) a history of gradual increase in symptoms and slowly progressing pulmonary fibrosis by chest roentgenogram compatible with a pneumoconiosis; (2) an appropriate history of exposure and a latency period of about 15 years before the onset of dyspnea and of roentgenographic changes; (3) analysis of open lung biopsy material revealing end-stage fibrosis and honeycombing, a moderate number of birefringent particles, and extremely high levels of a variety of zirconium compounds; and (4) no other potential cause of fibrosis. We conclude that zirconium should be considered a likely cause of pneumoconiosis and that appropriate precautions should be taken in the workplace.
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PMID:Zirconium compound-induced pulmonary fibrosis. 173 81

Between 1978 and 1988, three patients at our institution had an initial diagnosis of idiopathic pulmonary fibrosis but later were correctly diagnosed as having pulmonary-renal syndrome and microscopic polyarteritis. These cases involved elderly patients with progressive dyspnea and nonproductive cough, bilateral dry crackling rales, bilateral interstitial infiltrates evident on a chest roentgenogram, and restrictive findings on pulmonary function testing. In two patients, lung biopsy specimens were obtained, and an initial diagnosis of nonspecific pulmonary fibrosis was made. All three patients eventually had microhematuria and renal insufficiency. A revised diagnosis of small-vessel pulmonary-renal vasculitis was based on the demonstration of segmental necrotizing glomerulonephritis in renal biopsy specimens in two patients, thrombotic microangiopathy consistent with healed vasculitis on postmortem examination of the kidney in one patient, and subsequent detection of small-vessel vasculitis on review of the two lung biopsy specimens. Anti-neutrophil cytoplasmic antibodies with perinuclear staining on indirect immunofluorescence microscopy were positive in the two patients in whom determinations were performed. The clinical manifestations of vasculitis were notably scarce--no involvement of the skin, nervous system, or gastrointestinal tract; no episodes of fever; and minimal or absent musculoskeletal symptoms. These cases illustrate the importance of a high index of suspicion for the diagnosis of systemic vasculitis in elderly patients and the need to consider a vasculitis in the differential diagnosis of idiopathic pulmonary fibrosis, especially if an active urinary sediment is present.
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PMID:Pulmonary fibrosis as an unusual clinical manifestation of a pulmonary-renal vasculitis in elderly patients. 219 45

A 43-year-old woman was admitted to our hospital because of fever and dyspnea. She had facial rash, hair-loss, arthralgia, urinary casts, thrombocytopenia, lymphocytopenia, and deposits of IgG, IgM, C3 in the epidermis. SLE was diagnosed. She also had severe dyspnea, which gradually progressed. In spite of steroid pulse therapy and cyclophosphamide, she eventually died about two months after admission. Chest X-ray film showed bilateral ground-glass shadow and volume-loss. High-resolution CT of the lung showed marked high density area with airbronchogram in the posterior part and a gradually enlarging slight high density area in the anterior area. Pathological findings of the posterior area were atelectasis and dense fibrosis. The findings of the anterior area were collapsed alveoli and dilated alveolar ducts. These findings might suggest that alveolar collapse play a major role in the course of pulmonary fibrosis of this patient.
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PMID:[A case of acute lupus pneumonitis followed by high-resolution CT]. 221 22

With the general improvement in environmental controls in workplaces where asbestos is used, an increasing number of workers are seen who exhibit isolated pleural plaques. The question as to whether these are associated with respiratory impairment independently of parenchymal disease remains unresolved. The question was reinvestigated using quantitative gallium-67 lung scanning to take into account early parenchymal change not evident on the chest radiograph. We carried out a cross-sectional study of 110 construction insulators all currently at work. Overall, 58.2% had pleural abnormality, 52.5% pleural plaques only, and 5.5% diffuse pleural thickening as assessed from the PA chest radiograph. Compared with those without, those with any pleural abnormality had a decrease in FEV1 and FVC on average of 222 and 402 ml (p less than 0.05), and those with isolated pleural plaques, a decrease on average of 200 and 350 ml (p less than 0.05), after taking into account age, height, smoking status, and the presence of parenchymal abnormality as assessed by chest radiography and gallium uptake. The complaint of dyspnea with strenuous activities was also significantly related to the width and extent of chest wall pleural thickening (p less than 0.05), independently of parenchymal disease. This study suggests that the most common radiographic findings in asbestos-exposed, isolated pleural plaques are associated with a significant reduction in FEV1 and FVC, which cannot be attributed to the presence of radiographic and subradiographic pulmonary fibrosis.
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PMID:The relationship between respiratory impairment and asbestos-related pleural abnormality in an active work force. 202 35

Two cases of pulmonary alveolar proteinosis (PAP) with increased CEA were reported. Case 1 was a 62-year-old male with suspected pulmonary fibrosis, who was transferred to our hospital. Laboratory findings on admission revealed 31.3 ng/ml of CEA. Because he had severe dyspnea, lung biopsy was not carried out. His condition gradually deteriorated and he died of respiratory failure. Autopsy revealed he had PAP and no malignancy. Case 2 was a 48 year-old male referred to our hospital because of dyspnea. Serum CEA was 52.8 ng/ml. Microscopic examination of a transbronchial lung biopsy showed PAP. The level of CEA in bronchoalveolar lavage fluid was 151 ng/ml. Unilateral whole lung lavage was performed twice. With the improvement of chest X-ray findings, serum levels of CEA fell to normal level. The molecular weight of CEA in bronchoalveolar lavage fluid was 180,000. Immunochemical staining of CEA in lung revealed nonspecific findings.
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PMID:[Two cases of pulmonary alveolar proteinosis with increased CEA]. 223 73

A forty-eight year-old female with rheumatoid arthritis developed cough, sputum and dyspnea. Chest X-ray film demonstrated bilateral diffuse interstitial pneumonia and pulmonary fibrosis. Laboratory findings were as follows: ESR 29 mm/h, CRP 3.86 mg/dl, RA test (+), RAHA (-) and WBC 7200/mm3. Marked hypoxemia (po(2)45 Torr) was demonstrated by blood gas analysis. Asymptomatic pulmonary fibrotic lesions which preceded articular symptoms were identified on her previous chest X-ray films. Methylprednisolone-pulse therapy (1g/day, for 3 days) was repeated three times with a 2-week interval. The treatment rapidly improved both pulmonary symptoms and chest X-ray findings. Although the fibrotic shadows on chest X-ray did not completely disappear, her conditions have been maintained well under the treatment with prednisolone (10mg/day) plus D-penicillamine (200mg/day).
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PMID:[Methylprednisolone-pulse therapy in a patient with rheumatoid arthritis and diffuse interstitial pneumonia/pulmonary fibrosis]. 223 73

Methotrexate, an antifolate cytotoxic drug, is used in anticancer chemotherapy as well as an immuno suppressive in rheumatoid arthritis. It is responsible for numerous secondary effects, amongst which is a characteristic acute pneumonia known since 1969. This pneumonitis has been described in detail, up to the present time in 78 cases gathered in this review. The prevalence of this complication is estimated at around 7%. This pneumonia may occur whatever the age, indication for which methotrexate is prescribed, the route of administration of the product (including the intra-thecal route) and the dose. It includes dyspnoea, fever, (sometimes quite marked) and frequently an acute reversible respiratory failure. Radiologically the opacities are usually diffuse interstitial and symmetrical with a basal predominance with sometimes some confluence and occasionally a pleural reaction. In a small number of cases a transient mediastinal adenopathy has been described. Respiratory function tests show a rapidly developing restrictive syndrome accompanied by hypoxia and hypocapnia. Broncho-alveolar lavage is characterised by hypercellularity with a frank and apparently transitory lymphocytosis. Histologically the most frequent lesion sighted is an extensive acute granulomatous reaction with or without oedema. Most often the outcome is favourable (75% of cases). However 6 deaths due to respiratory failure have been reported. Even though there has not been any formal test, steroid therapy in high dosage seems to accelerate recovery. Progress to an irreversible pulmonary fibrosis is possible but rare. The mechanism of this drug related acute pneumonia is not known but would seem to resemble that of other granulomatosis. Besides this rapidly progressive pneumonitis, methotrexate is responsible for a very small number of cases of severe pulmonary oedema and of acute painful pleurisies.
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PMID:[Pneumopathy caused by methotrexate]. 225 35

Organic dust toxic syndrome is a term recently coined to describe a noninfectious, febrile illness associated with chills, malaise, myalgia, a dry cough, dyspnea, headache and nausea which occurs after heavy organic dust exposure. Organic dust toxic syndrome shares many clinical features with acute farmer's lung and other forms of hypersensitivity pneumonitis, including the presence of increased numbers of neutrophils in bronchoalveolar lavage. However, organic dust toxic syndrome differs from acute hypersensitivity pneumonitis in several respects: the chest X-ray does not show infiltrates, severe hypoxemia does not occur, prior sensitization to antigens in the organic dust is not required and there are no known sequelae of physiological significance, such as the recurrent attacks and the pulmonary fibrosis which may be seen with chronic hypersensitivity pneumonitis. Organic dust toxic syndrome is thought to be much more common than farmer's lung. It is important for clinical and investigational purposes that organic dust toxic syndrome be distinguished from acute farmer's lung.
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PMID:Organic dust toxic syndrome: an acute febrile reaction to organic dust exposure distinct from hypersensitivity pneumonitis. 226 97

In a 39-year-old patient with chronic progressive idiopathic pulmonary fibrosis, the genetic aspects, course and therapeutic possibilities of the disease are discussed. In February, 1987, the English-born patient, Anthony V., attended for initial examination on account of progressive dyspnoea, on which occasion radiology and pulmonary function analysis revealed advanced pulmonary fibrosis. The patient's family history revealed a familial genesis, since both his father (?) and his sister had died of this disease. A comparative of the patient's chest films with original chest films of his sister revealed almost identical findings. Within the previous twelve months, follow-up examinations done on A.V. revealed an increase in the restrictive component (reduction of vital capacity from 2,400 ml to 1,500 ml), development of partial respiratory failure at rest, and global respiratory failure in response to mild ergometric exercise despite intermittent high-dose steroid administrations superimposed on long-term, low-dose steroid therapy. The unfavourable evolution observed over the past 12 months is underscored by an increase in mean pulmonary arterial pressure from 18 mmHg initially to a present 34 mmHg at rest, and 46 mmHg under submaximal ergometric loading. The only option still left to the patient is the possibility of a lung transplantation, which - probably initially unilateral - is scheduled to be carried out in the near future at the Chest Surgery Department of the Medical University at Hannover.
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PMID:[Idiopathic lung fibrosis]. 236 51


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