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Query: UMLS:C0034069 (pulmonary fibrosis)
 7,050 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The supply of donor organs remains extremely limited, and improved methods of maintaining the lungs of potential donors to allow for transplantation must be developed. Currently the upper limit of donor lung ischemic even with our "best" preservation techniques is approximately 4 to 6 hours. Improved methods for preservation will increase the supply of suitable lungs and will considerably simplify the logistics of transplantation just as has occurred with liver transplantation. Efficient use of donor organs remains of paramount importance. We recently performed two single-lung transplants utilizing lungs from one donor. Likewise, there is no reason why a lung could not be sent to another center for transplantation if the harvesting group uses only one lung. Sufficient progress has been achieved to date to warrant continued application of lung transplantation for end-stage pulmonary disease. With increasing experience, one can anticipate refinement of techniques and broader application of these procedures. Single lung transplantation, initially restricted to patients with end-stage pulmonary fibrosis, has now been successfully applied to patients with emphysema, pulmonary hypertension, and other conditions. Although transplantation currently can offer real benefit only to a limited number of persons, it serves to create hope for many others. An additional benefit may prove to be the interest and attention that transplantation focuses on patients with end-stage lung disease and on the pathophysiology of chronic respiratory failure. Knowledge gained may ultimately result in the prevention of many of the disorders for which lung transplantation currently offers the only hope.
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PMID:Lung transplantation. 268 Feb 89

Systemic sclerosis is frequently complicated by fibrosing alveolitis although clinical and radiological abnormalities are not usually apparent until the lung disease is well established. The aim of this study was to investigate pulmonary involvement in systemic sclerosis by thin section CT scan, bronchoalveolar lavage (BAL) and 99mTc-DTPA clearance studies, and assess the value of these tests in defining pulmonary abnormalities in patients with a normal chest radiograph. Patients were divided into those with an abnormal chest radiograph (Group I, n = 14) and those with a normal chest radiograph (Group II, n = 16). CT scans were abnormal in all patients in Group I and 7 of 16 (44%) in Group II. BAL inflammatory cell counts were raised in all 12 (100%) patients studied in Group I and 11 of 15 (73%) in Group II. There was no difference in the type of inflammatory cells observed between the two groups. 99mTc-DTPA clearance was faster than normal controls in ten of 14 patients (71%) in Group I and seven of 15 (47%) in Group II and correlated with carbon monoxide transfer factor (P less than 0.05). Lung biopsies were performed on nine patients in Group I and three in Group II all of whom had abnormal CT scans. Fibrosing alveolitis was confirmed in every case. Group II biopsies could not be distinguished from Group I biopsies; both showed fibrosis as well as inflammation suggesting that pulmonary fibrosis is an early abnormality in systemic sclerosis. Our results indicate that CT scans, BAL and 99mTc-DTPA are frequently abnormal in asymptomatic patients with systemic sclerosis who have normal chest radiographs. When the CT scan is normal abnormalities of BAL and/or 99mTc-DTPA (99mTechnetium diethylenetriamine pentacetate) clearance may indicate lung disease at a still earlier stage. This observation requires further investigation.
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PMID:Pulmonary involvement in systemic sclerosis: the detection of early changes by thin section CT scan, bronchoalveolar lavage and 99mTc-DTPA clearance. 269 36

Thoracoscopy was performed under local anesthesia in 419 patients suffering from a diffuse lung disease. In 85% of the cases diagnosis was clarified by thoracoscopy. All other cases were confirmed by means of an open lung biopsy. The best results were obtained in sarcoidosis of the stages II and III, the sensitivity being 0.98. Tumour-conditioned diffuse lung diseases were clarified in 88% of the cases; proof of an interstitial pulmonary fibrosis or interstitial pneumonia was established in 85% of the patients. Results regarding histiocytosis X were poor: thoracoscopic-bioptic proof was successful in only 42% of the patients. In 419 examinations we only detected a severe complication (air embolism). Drainage times were on the average between 4 and 5 days. On the whole, the method was characterised in the field of diagnosis of diffuse lung diseases by a high degree of sensitivity and satisfactory specificity. Both in respect of the invasiveness of the examination and its sensitivity it occupies an intermediate position between peripheral bronchoscopically obtained biopsy and surgical open lung biopsy, representing a valuable extension of the diagnostic instrumentarium if the indication is carefully considered.
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PMID:[Thoracoscopy in diffuse lung diseases]. 271 51

One hundred forty-one retired Greek mariners were examined radiologically for asbestos-related lung disease. Thirty-eight (27%) had small opacities classified as ILO category 1/0 or more; 37 (26%) had radiologic evidence of pleural lesions; 17 (12%) had both parenchymal and pleural lesions; and a total of 58 (41%) had one or more radiologic findings of asbestos-related lung disease. In discriminant analysis, duration of maritime employment was predictive of pleural lesions, but the association was not statistically significant (one-tail, p = .16). The prevalence of pleural lesions was also higher among sailors than among officers, and this association was statistically significant (one-tail, p = .05). In this group, none of the occupational variables studied (age, duration of maritime employment, and rank) was related to lung fibrosis. After controlling by multiple regression for mutual confounding effects, suggestive negative associations for the presence of pleural lesions were found with FVC (one-tail, p = .13) as well as with FEF25% (one-tail, p = .09) and FEF50% (one-tail, p = .07). By contrast, no association was found between pulmonary fibrosis and any of the respiratory volumes. The results of this study suggest that mariners may present evidence of asbestos-related disease after many years from onset of exposure on ships.
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PMID:Asbestos-related chest X-ray changes among Greek merchant marine seamen. 274 57

We studied the chest radiographs of 34 consecutive patients with diffuse pulmonary fibrosis to determine the presence of tracheomegaly and to follow its progression with time. Patients had been identified by a computer search of medical records. We measured the internal transverse diameter of the trachea 2 cm above the top of the aortic arch on erect posteroanterior chest radiographs. Transverse diameters greater than 25 mm in men and 21 mm in women were considered indicative of tracheomegaly. Pulmonary-function tests, available in 30 of the 34 patients, showed restrictive lung disease. The transverse tracheal measurements were compared with the cause of fibrosis, severity of restriction, duration of illness, and other clinical variables. Tracheomegaly was present in 10 (29%) of the patients, including four with fibrosing alveolitis, four with sarcoidosis, and two with chronic progressive histoplasmosis. In seven of these patients, serial radiographs showed that the tracheal dilatation had progressed with time. Nine of 24 patients without tracheomegaly also had progressive increase in transverse tracheal diameter over time. Of the 10 patients with tracheomegaly, pulmonary-function tests were available in eight and showed moderate restrictive lung disease in six and severe restrictive lung disease in two. The duration of illness was 3-6 months in two patients, 10-22 years in five patients, and not recorded in three patients. Chronic cough and repeated respiratory infections were slightly more common in those patients with tracheomegaly than in those without. These data suggest that tracheomegaly develops as a complication of diffuse pulmonary fibrosis in patients who have at least moderate restrictive lung disease and prolonged illness, and it may have some association with chronic cough and repeated respiratory infection.
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PMID:Acquired tracheomegaly in adults as a complication of diffuse pulmonary fibrosis. 278 56

A study was initiated to determine whether alveolar macrophages from patients with collagen vascular diseases but free of pulmonary symptoms were spontaneously activated and whether they released various mediators related to the pathogenesis of pulmonary fibrosis. Alveolar macrophages obtained by bronchoalveolar lavage from 32 patients with proved collagen vascular disease but no evidence of lung disease were compared with those from 10 patients with collagen vascular disease with interstitial lung disease (CVD-ILD) and from 10 healthy controls. The total number of alveolar macrophages did not differ between patients with collagen vascular disease and controls but were substantially increased in the CVD-ILD group. Alveolar macrophages from 31 of the 32 patients with collagen vascular disease and from all 10 in the CVD-ILD group had at least one criterion of activation. Neutrophil chemotactic activity was detected in supernatants from alveolar macrophage culture in 23 of the 32 patients with collagen vascular disease and from nine of the 10 in the CVD-ILD group; fibronectin secretion by alveolar macrophages was increased in 12 of the 32 patients with collagen vascular disease and in nine of the 10 in the CVD-ILD group. Furthermore, alveolar macrophages from 20 of the 32 patients with collagen vascular disease and four of the 10 CVD-ILD patients spontaneously released increased amounts of superoxide anion. Thus alveolar macrophages were spontaneously activated in a high proportion of patients with collagen vascular disease.
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PMID:Activated alveolar macrophages in subclinical pulmonary inflammation in collagen vascular diseases. 283 61

The radiologic appearance of atypical cardiogenic pulmonary edema (ACPE) is presented in 10 cases admitted from 1983 to 1985, with age ranges from 74 to 89, and with diagnosis of ischemic heart disease, with myocardial infarction in 50% of them. Clinically they had asthenia, adynamia and anorexia in 80%, cough and weight loss in 50%. All of them had tachycardia, pulmonary rales and 50% pericardial rub. ECG showed in 80% anterior subepicardial ischemia, 60% posteroinferior subepicardial ischemia, 60% bifascicular block, and 50% left anterior fascicular block. Chest films were interpreted at first as pulmonary fibrosis in 90% of the cases with superior lobe involvement in 50%. Heart enlargement was present in 50%. A chronic lung disease was disclosed on clinical and pulmonary physiological grounds. It is concluded that asthenia, adynamia and anorexia were atypical manifestations of heart failure in the elderly. Silent myocardial infarction was observed in half of our patients and it was complicated with pericardial involvement in 50%. Irregular distribution of fluids in pulmonary edema was attributed to anatomic changes in elder lung. These atypical behaviour of pulmonary edema, has been misinterpreted on radiologic basis with pulmonary infection, tumours, metastasis or fibrosis. Those radiologic changes disappeared or improved in 72 hrs. with treatment of left ventricular failure.
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PMID:[Radiologic characteristics of cardiogenic pulmonary edema in the elderly]. 296 66

When evaluating diffuse lung infiltrates, the clinician should place special emphasis on the acuity of symptoms, nonpulmonary complaints and findings, environmental exposures, and risk factors for immunosuppressive diseases. Certain radiographic features, such as the distribution of opacities, hilar adenopathy, Kerley-B lines or pneumothorax, or pulmonary function tests demonstrating air flow limitation also narrow the differential diagnosis. One can direct the subsequent workup based on the narrowed differential diagnosis, the pace of disease, the activity of the ongoing inflammatory-immune process, and the age, overall medical condition, and wishes of the patient. Unless a specific diagnosis (for example, hypersensitivity pneumonitis, the treatment of which is withdrawal of the offending agent) can be made, therapy of noninfectious diffuse lung disease is quite unsatisfactory. Immunosuppressive therapy is indicated to arrest the active inflammatory process with the hope that objective signs of improvement will occur after a 3- to 12-month course. Important areas of basic research in pulmonary fibrosis include cell-cell and cell-matrix interactions in the lung interstitium and delineation of fibroblast biology and cytokine-mediated lung connective tissue pathology. More successful therapies will probably evolve from better understanding of the molecular and cellular biology of the lung fibrogenic process.
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PMID:Approach to the patient with diffuse lung disease. 304 56

Dendriform pulmonary ossification is a rare entity associated with chronic lung disease that is almost invariably discovered as an incidental finding at autopsy. Antemortem chest roentgenograms of patients with dendriform pulmonary ossification are often interpreted as pulmonary fibrosis and/or bronchiectasis. Radiographic and pathologic findings in two cases are described herein.
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PMID:Dendriform pulmonary ossification. 311 14

Selected patients with terminal lung disease have been managed effectively by lung transplantation. Strict selection criteria for donors and recipients, attention to technical detail, and avoidance of perioperative corticosteroids increase the likelihood of success. The underlying pulmonary disease determines the appropriate procedure. Single-lung transplantation is most appropriate for patients with pulmonary fibrosis. Patients with emphysema or septic pulmonary disease who have adequate or recoverable cardiac function can be well served by double-lung transplantation. However, such patients are still treated in some centers by combined heart-lung transplantation. Patients with right-heart failure secondary to vascular or parenchymal pulmonary disease are best managed by combined heart-lung transplantation. Donor availability, airway healing, and diagnosis of rejection remain significant problems and are the focus of experimental and clinical investigation in many centers.
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PMID:Status of lung transplantation. 328 63


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