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

Over the past ten years there has been fundamental progress in molecular biology, i.e. concerning the structure and function of genes. The understanding and diagnosis of several diseases, in particular those of the respiratory system, have been profoundly affected and changed. For example alpha-1-antitrypsin deficiency and the emphysema which results have now been dissected down to a molecular level and characterised by anomalies of certain critical portions of the gene coding for this protein. The same thing is found in cystic fibrosis where, thanks to recent technical progress, it is now possible to make a positive diagnosis in most unaffected carriers. The importance of molecular biology in lung cancer is equally established, and in small cell lung cancer one can already isolate a sub group of cancers presenting with an abnormal amplification of the c-myc oncogene. Finally, the role of inflammatory cells, in particular macrophages, in pulmonary fibrosis is best understood by studying the expression by macrophages of the genes coding for mediators which alter the replication of fibroblasts.
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PMID:[Application of molecular biology techniques to pneumology]. 217 33

The techniques and indications for lung transplantation have evolved significantly in the past 6 years. We initially restricted single-lung transplantation to patients with pulmonary fibrosis and developed the double-lung transplantation procedure for patients with emphysema or cystic fibrosis. However, with the double-lung procedure, a 25% mortality rate resulted from ischemic complications at the tracheal anastomosis. The technique was altered to incorporate bilateral bronchial anastomosis, with a resulting reduction in airway complications. The double-lung transplantation procedure continued to have significant drawbacks, including intraoperative and postoperative hemorrhage, and cardiac complications due to prolonged cardiopulmonary bypass, ischemic cardiac arrest, and extensive manipulation of the heart. These problems recently have been addressed with a much simplified procedure incorporating a bilateral transverse thoracosternotomy, replacement of the right lung without cardiopulmonary bypass, and replacement of the left lung with or without a short period of partial bypass. This procedure has been successfully used for emphysema and for cystic fibrosis. The technique of single-lung transplantation also has been simplified and indications have been expanded to include selected patients with emphysema and with primary pulmonary hypertension. With single-lung transplantation, each of the lungs from a donor has been successfully used for a separate recipient on several occasions, improving the supply of available donor organs.
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PMID:The evolution of techniques and indications for lung transplantation. 220 16

Since January 1988, the Bordeaux group has performed 15 transplantations for lung disease: 9 heart-lung transplants, 1 heart + left lung, 1 double lung, 2 right lungs and 2 left lungs. The transplantations were performed for pulmonary emphysema (10 cases), pulmonary artery hypertension (2 cases), cystic fibrosis (1 case), pulmonary fibrosis (2 cases). Cardiopulmonary transplantation was not always performed because of associated heart failure but sometimes because of large intrahilar adenopathy or intractable bronchial infection. Pulmonary transplantation is recommended on the right side in cases of pulmonary fibrosis. One patient died postoperatively (ischaemia of the transplant). Four others died during the 2nd and 3rd months from poorly defined but probably infectious pulmonary syndromes. The tracheobronchial patency of the 10 survivors was 80% or 100% of the predicted value. The respiratory functional result was excellent in the short and intermediate term. Specific difficulties essentially consisted of pleural symphyses, hilar adenopathy, bronchial infection, steroid dependence of certain subjects, the difficulty of identifying the cause and treating lung opacities during the 2nd and 3rd months.
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PMID:[Lung and heart-lung transplantation in respiratory tract diseases. Evaluation and development of the indications based on our first 15 cases]. 258 94

Chronic obstructive pulmonary disease (COPD) is equated with chronic bronchitis and emphysema as one disease entity. In COPD airflow limitation is relatively persistent--unlike asthma. Tests for "small-airways disease" form no part of routine practice, for their accuracy in detecting pathological change is debatable. The proteolytic theory of the pathogenesis of emphysema highlights the role of neutrophil elastase, antielastases, oxidants, antioxidants, and thus of potential new treatments. Clinical features of COPD include breathlessness, cough, and sputum, with airflow obstruction and lung hyperinflation. The differential diagnosis includes bronchiectasis, cystic fibrosis, and pulmonary hypertension, but pulmonary fibrosis, etc., is distinguished by radiological infiltrates. Plain chest radiography cannot reliably diagnose emphysema in life, but a new method measuring lung density from the computed tomographic (CT) scan allows location, quantitation, and diagnosis of emphysema (defined by enlargement of distal air spaces) in humans in life. "Pink puffers" with breathlessness, hyperinflation, mild hypoxemia, and a low PCO2 are contrasted with "blue bloaters" with hypoxemia, secondary polycythemia, CO2 retention, and pulmonary hypertension and cor pulmonale. Antismoking measures are a major aim in management. A bronchodilator regimen combining a slow-release oral theophylline with an inhaled beta 2-agonist, ipratropium, and high-dose inhaled steroids is proposed because even modest improvement in obstruction can help these patients. In acute exacerbations with purulent sputum, antimicrobials against Streptococcus pneumoniae and Hemophilus influenzae are used with controlled oxygen therapy aiming to keep the arterial PO2 over 50 mm Hg without the pH falling below 7.25. Influenza prophylaxis is recommended, but pneumococcal vaccination remains debatable. Chronic under-nutrition in "emphysema" implies controlled trials of feeding regimens--but these remain to be assessed. Long-term oxygen therapy is the only treatment known to prolong life in blue bloaters, and oxygen concentrators and transtracheal oxygen delivery are discussed. Pulmonary vasodilators (e.g., beta 2-agonists, hydralazine, nifedipine, angiotensin-converting enzyme [ACE] inhibitors, etc.) have not yet been proved to provide long-term reduction in pulmonary arterial pressure. Blue bloaters have severe nocturnal hypoxemia in rapid eye movement (REM) sleep that is corrected by oxygen or the investigational drug almitrine.(ABSTRACT TRUNCATED AT 400 WORDS)
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PMID:Chronic obstructive pulmonary disease. 304 40

Emphysema, chronic bronchitis, asthma, and cystic fibrosis are often cited as examples of chronic airways obstruction, while sarcoidosis is typically restrictive. Approximately 15 percent of sarcoidosis patients, however, have airways obstruction clinically characterized by wheezing with granulomatous involvement of airways. Since the majority have Stage IV disease by chest radiograph, their lungs usually have honey-combining with pulmonary fibrosis, adhesions, cavities, and mediastinal distortion.Patients had a mixed ventilatory defect, but obstruction of large airways was present as shown by decreased specific airways conductances. Small airways obstruction was also present as shown by low instantaneous flows at the terminal portion of the maximum expiratory flow-volume curve and diminished helium response of this curve. The closing volume, however, was not very sensitive. Radioactive xenon washout from ventilation lung scans and N2 washout from the lungs were prolonged in patients with worse disease.The authors conclude that the obstructive type of physiologic pattern is more frequent than recognized in sarcoidosis, which like that of cystic fibrosis has some restrictive element and is characterized by poor reversibility to bronchodilators. A trial period of beclomethasone dipropionate aerosol was not helpful in two patients. Relief of this distressing airways obstruction continues to pose a challenging problem in management.
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PMID:Chronic airways obstruction in pulmonary sarcoidosis: its poor response to bronchodilators. 742 Apr 39

Progress over the last 40 years has greatly reduced morbidity and mortality in the constantly changing field of thoracic surgery. The first part of this review focuses on current indications and limitations in lung surgery. Technical procedures for pneumonectomy, lobectomy, bronchial resection and conservative surgery are well established. Although major respiratory or cardiac failure still limit indications bronchogenic cancer extension is no longer a contraindication. Exeresis after 70 years of age is not an exception. Surgery for non-small cell lung cancer has given promising results with a 5-year survival rate of 60-80% for patients in stage I and II. For stage III, two recent comparative studies have demonstrated the effectiveness of preoperative adjuvant chemotherapy which should logically be proposed with or without radiotherapy in patients with resectable tumours. Surgical removal of lung metastases and mesotheliomas has also made considerable progress. Unfortunately, except for therapeutic trials, exeresis of small cell lung cancer does not provide any beneficial effect and cannot be proposed. Indications for surgery in patients with chronic obstructive pulmonary disease however has been quite successful and now goes beyond classical exeresis of large compressive bullae. In many situations patients with diffuse emphysema can benefit from surgical reduction in lung volume before proposing transplantation. Lung transplantation is indicated for pulmonary fibrosis, pulmonary vascular disease and obstructive lung pulmonary disease with an overall survival rate of 50% at 5 years and 43% at 6 years. The rate of successful bilateral lung transplantation for cystic fibrosis remains to be determined.
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PMID:[New techniques in thoracic surgery. I]. 756 9

Air travel can cause severe respiratory decompensation in a patient with advanced lung disease due to high altitude hypoxemia. We report our experience in flying 21 patients with advanced lung disease to a medical center remote from Israel for lung transplantation or pulmonary thromboendarterectomy (PTE). All patients had severe lung disease with marked hypoxemia (PaO2, 40 to 59) and 16 had significant pulmonary hypertension. Nine patients (with emphysema and pulmonary fibrosis) required single lung transplant, four (with cystic fibrosis and emphysema) required double-lung transplant, six (with primary or secondary pulmonary hypertension) required heart-lung transplant, and two (with major vessel pulmonary thrombosis) required PTE. All patients were flown by commercial aircraft to centers located 2,634 to 13,181 km away from Israel. Length of flight was between 4 and 21 h. Patients were given oxygen supplementation during the flight and were monitored by portable oximeters. All but three patients were hemodynamically stable and 19 of them were escorted by physicians. All but one hemodynamically unstable patient who died on board arrived safely at their destinations. We conclude that with careful preparation, sufficient oxygen supply, oximetric monitoring, and medical escort, almost any patient with severe lung disease can travel by air to any necessary destination.
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PMID:The safety of air transportation of patients with advanced lung disease. Experience with 21 patients requiring lung transplantation or pulmonary thromboendarterectomy. 758 32

Heart-lung transplantation is a complex, expensive, and resource-intensive procedure. It is performed more often where there is coexistence of end-stage pulmonary disease with advanced cardiac disease that is not secondary to pulmonary hypertension (e.g., some forms of congenital heart disease, some situations with Eisenmenger's syndrome, and possibly the coexistence of end-stage heart and end-stage lung disease). Available data indicate that some disease states are associated with a higher probability of successful outcome after HLT than are others. Objectively validated and reliable patient selection criteria cannot be obtained from the current medical literature. Expert opinion provided to OHTA contained varying degrees of detail and uniformity with respect to patient selection (Tables 11 and 12). While most agree that "irreversible cardiopulmonary disease" is an indication for this procedure, there are no published data that have provided details as to how patients so designated were selected as candidates for HLT. For example, for chronic obstructive pulmonary disease (emphysema), neither expert opinion nor published studies of HLT provided data indicating the stage of the disease at which a decision to transplant is reasonable and objectively justifiable. A similar lack of detail for cystic fibrosis, bronchiectasis, pulmonary fibrosis, sarcoidosis, asbestosis, and other diseases was noted. Thus, there are insufficient data regarding transplant recipients to permit the development of objective criteria for a threshold for HLT in the various disease states. The circumstances are more problematic regarding suggested contraindications for HLT. While objective survival rates obtained from registry data indicated that survival bore little relationship to recipient age, various opinions provided to OHTA specified a maximum age for HLT candidates, ranging from under 45 years to 60 years. Adverse factors and contraindications, such as "other significant disease," "recurrent pulmonary emboli," "hepatic disease," "infection," and "insulin-dependent diabetes" were nonspecific and unsupported by published, objective evidence. More ambiguous contraindications included "psychiatric illness," "poor family support structure," "history of noncompliance with medical regimens," and lack of "emotional stability." While psychiatric, psychologic, or sociologic evaluations of potential HLT recipients undeniably have relevance to patient selection, use of such criteria should be supported at least by evidence of generally consistent application across the transplant community. Of note was the recommended requirement that the recipient possess "adequate financial resources," which implies that the benefits of HLT are not expected to be equitably provided to the public.(ABSTRACT TRUNCATED AT 400 WORDS)
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PMID:Institutional and patient criteria for heart-lung transplantation. 771 18

Between November 1992 and May 1994 we performed 10 single and 5 double lung transplants in patients with end-stage lung diseases due to lymphangioleiomyomatosis (4), cystic fibrosis (3), pulmonary hypertension (3), pulmonary fibrosis (3) and chronic obstructive lung disease (2). In the 13 patients (87%) surviving for median 245 (19-567) days, FEV1 improved from median 640 ml to 1410 ml and the 12-minute walk distance from median 315 to 1100 meters. 10 patients (77%) enjoy a good or even excellent quality of life. 2 patients died 11 and 62 days postoperatively, due to multi-organ failure and invasive pulmonary aspergillosis respectively. The main postoperative problems are fungal and cytomegalovirus infections and chronic rejection in the form of bronchiolitis obliterans. In Switzerland as elsewhere, lung transplantation has become an established modality for the management of end-stage diseases of the lung and pulmonary circulation.
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PMID:[Isolated lung transplantation--evaluation of patients and initial results]. 773 52

Lung transplantation is a successful alternative treatment for a variety of end-stage lung diseases. The first 20 lung transplants performed in Louisiana between November 1990 and July 1994 are reported from Ochsner Foundation Hospital. Transplant procedures included 1 heart-lung, 11 bilateral sequential lung, and 8 single-lung transplants in 8 males and 11 females (1 retransplantation). The average age was 38 years (range 7-60), and the median waiting time was 34.5 days (range 1-329). Indications for transplant included emphysema, pulmonary fibrosis, pulmonary hypertension, cystic fibrosis, bronchiectasis, and bronchiolitis obliterans. Overall 1-year and 3-year survival were 65.0% and 58.5%, respectively. Infection was the major cause of morbidity and mortality. Rejection episodes were observed but treated successfully in all 20 patients. Lung transplantation has proved to be a successful treatment for a variety of severely limiting and terminal pulmonary conditions for patients in our state.
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PMID:Lung transplantation in Louisiana: report of the first twenty lung transplants performed in the state. 789 Oct 1


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