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)

Between August 1986-July 1994 45 1 patients with chronic hypoxaemia were referred to us for evaluation. 315 of them (70%) were qualified for LTOT according to national guidelines. There were 189 pts with COPD (60%), 40 with late sequelae of pulmonary tuberculosis (TB), 21 with interstitial pulmonary fibrosis (IPF), 15 with bronchiectasis (BE), 15 with severe kyphoscoliosis (KS) and 35 with other disease leading to chronic respiratory failure. All patients received oxygen from an oxygen concentrator and have been regularly followed-up. The best survival rate in patients followed up for at least 3 years was observed in KS (68%) and COPD pts (50%). The worst survival was seen in BE (9%) and IPF (21%). 183 pts died during the follow-up and in 3 pts (1%) LTOT was withdrawn. The most frequent cause of death were either acute (58%) or chronic (21%) cardiorespiratory failure.
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PMID:[Eight years of experience in Warsaw with domiciliary long term oxygen therapy]. 899 52

Diffusion capacity for carbon monoxide of the lung (DLCO) before-and-after exercise test and 5 routine pulmonary function tests were conducted in 16 patients with diffuse pulmonary fibrosis (DPF), 19 patients with interstitial pneumonia (IP), 17 patients with COPD, and 22 normal subjects. The data showed: in normal subjects the DLCO after exercise increased significantly compared with before (P < 0.001). But in DPF group the DLCO before exercise was below the normal range, and it went down further after exercise, the decreasing rate being 17.95% (P < 0.001). The DLCO in IP and COPD groups did not significantly change before and after the exercise. Two cases of interstitial pneumonia, who had a reduced DLCO after exercise, were followed up for 0.5-1.0 year, and both patients developed into DPF by that time. The results suggest that the DLCO measure before and after exercise test may have important value for DPF diagnosis, and a reduced DLCO after exercise test in IP patients may warn the development from IP into DPF.
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PMID:[Diagnostic value of exercise diffusion capacity test on diffuse pulmonary fibrosis]. 938 39

The lungs are a delicate interface between the atmosphere and our bodies across which oxygen diffuses from the air we breathe to the blood which carries oxygen to the cells and mitochondria. In healthy lungs at sea level where there is a surfeit of oxygen, this process occurs easily, whereas, in lungs with disease it becomes a task which may not be fully successful and hypoxemia may ensue or worsen. At high altitude where the barometric pressure (Pb) and thus the supply of oxygen is lower, the job of getting oxygen to the blood, even in the healthy lung is more difficult, and in the diseased lung it may be impossible. This presentation will review the lungs' responses to high altitude, with emphasis on the abnormal. Both acute and chronic responses of patients with pre-existing lung disease will be reviewed. Pulmonary diseases encountered at high altitude in previously healthy people, such as high altitude pulmonary edema and chronic mountain sickness will be touched on only as they pertain to other patients. Pre-existing lung disease (with and without hypoxemia at sea level) such as obstructive lung diseases (asthma, COPD, emphysema), and restrictive lung diseases (sarcoid, asbestosis, interstitial pulmonary fibrosis) will be discussed in terms of gas exchange, lung mechanics, and treatment at high altitude. Disorders of ventilatory control; e.g., obesity-hypoventilation syndrome and sleep apnea, may present formidable problems, and guidelines for their treatment will be discussed. Infectious lung diseases; e.g., pneumonia, cystic fibrosis, and pulmonary vascular disorders such as chronic mountain sickness, primary pulmonary hypertension, and congenital absence of the pulmonary artery are important disorders that require special attention because of the accentuated hypoxic pulmonary vascular response encountered at high altitude. The purpose therefore, is to provide the medical practitioner with the insight into prevention, recognition, and treatment of pulmonary problems encountered specifically at high altitude, as well as guidance on how best to advise patients with lung disease who want to fly in airplanes and/or ascend to high altitude for work or pleasure.
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PMID:Lung disease at high altitude. 1063 92

In the last 2 years, four hospitals performmed cooperatively single-lung transplantation for 3 patients, 1 left lung and 2 right lung. The patients had pulmonary fibrosis, COPD with cancer or tuberculosis, infection in some extent before transplantation. The patients died from severe infection 9, 48 and 43 days after the operation. We discussed the selection of donors and recipients, operative procedures, postoperative management, especially monitoring, differential diagnosis and treatment of infection and rejection. HLA compatibility and transbronchial lung biopsy were important to the success of lung transplantation. We believe that rejection and infection are important causes of short-term death that should be given more attention.
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PMID:[Experience and lessons of lung transplantation]. 1067 54

Poland's Institute of Tuberculosis and Lung Diseases oversees 49 provincial clinics, which provide and monitor LTOT for patients with COPD, interstitial pulmonary fibrosis, and other pulmonary conditions. Because of limited resources, eligibility for LTOT is fairly strictly defined, and LTOT equipment is distributed to and retained only by nonsmoking patients who continue to demonstrate need of the equipment (i.e., those who have ongoing hypoxemia that can benefit from LTOT). This national LTOT system provides a large, nonselected population suitable for LTOT research, and recent studies have produced important data regarding survival, pulmonary hemodynamics, and the effect of withdrawing LTOT from patients whose oxygenation has recovered to above the LTOT qualification level of PaO2 < or = 55 mm Hg.
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PMID:A nationwide system of long-term oxygen therapy: the Polish experience. 1077 95

Lung cancer is associated with smoking and age, both of which are associated with comorbidity. We evaluated the impact of comorbidity on lung cancer survival. Data on 56 comorbidities were abstracted from the records of a cohort of 1,155 patients. Survival effects were evaluated with Cox regression (outcome crude death). The adjusted R(2) statistic was used to compare the survival variation explained by predictive variables. No comorbidity was observed in 11.7% of patients, while 54.3% had 3 or more (mean 2.97) comorbidities. In multivariate analysis, 19 comorbidities were associated with survival: HIV/AIDS, tuberculosis, previous metastatic cancer, thyroid/glandular diseases, electrolyte imbalance, anemia, other blood diseases, dementia, neurologic disease, congestive heart failure, COPD, asthma, pulmonary fibrosis, liver disease, gastrointestinal bleeding, renal disease, connective tissue disease, osteoporosis and peripheral vascular disease. Only the latter was protective. Some of the hazards of comorbidities were explained by more directly acting comorbidities and/or receipt of treatment. Stage explained 25.4% of the survival variation. In addition to stage, the 19 comorbidities explained 6.1%, treatments 9.2%, age 3.7% and histology 1.3%. Thirteen uncommon comorbidities (prevalence <6%) affected 21.2% of patients and explained 3.5% of the survival variation. Comorbidity count and the Charlson index were significant predictors but explained only 2.5% and 2.0% of the survival variation, respectively. Comorbidity has a major impact on survival in early- and late-stage disease, and even infrequent deleterious comorbidities are important collectively. Comorbidity count and the Charlson index failed to capture much information. Clinical practice and trials need to consider the effect of comorbidity in lung cancer patients.
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PMID:Impact of comorbidity on lung cancer survival. 1251 1

Lung transplantation is a relatively new field in solid organ transplantation. We present our early experience with the first 70 cases at the Rabin Medical Center during the years 1997-2003. Forty seven patients underwent single lung, eight double lung and eight heart-lung transplantations. The patients treated included 49 men and 21 women aged 5-66 years. There were 26 cases with emphysema COPD. 30 patients with pulmonary fibrosis. 5 patients with pulmonary hypertension/Eisenmenger and 9 patients with cystic fibrosis and bronchiectasis. Although early results (1997-1999) showed 1 and 3 year survival of only 50%, in the last 3 years (2000-2003), survival reached 84% and 82% at 1 and 3 years respectively. Improvement in the success rate is due to better patient selection, new immunosuppressive regimen and, most importantly, excellent teamwork. We conclude that lung transplantation is a viable option for selected patients with end-stage lung disease.
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PMID:[Lung and heart-lung transplantation in Rabin medical center: early experience with 70 cases]. 1474 77

The purpose of this study was to explore the relationship between diagnosis and the cost-effectiveness and cost-utility of lung transplantation. A microsimulation model was used, based on empirical data from the Dutch lung transplantation program, collected between 1991 and 1999. We assessed life-years, quality-adjusted life-years, and costs with and without transplantation for the diagnostic categories alfa-1 antitrypsin deficiency, COPD/emphysema, bronchiectasis, primary and secondary pulmonary hypertension, cystic fibrosis, and pulmonary fibrosis. Alfa-1 antitrypsin deficiency and bronchiectasis had the highest survival gain. Secondary pulmonary hypertension and pulmonary fibrosis had the lowest survival gain and the lowest gain of quality-adjusted life-years. As compared with COPD/emphysema, alfa-1 antitrypsin deficiency, bronchiectasis, and CF had 25%, 40% and 19% more favorable cost-effectiveness ratios, respectively. Cost-utility ratios varied less, with values of -7%, -14% and -11% for alfa-1 antitrypsin deficiency, bronchiectasis, and primary pulmonary hypertension, respectively, compared with COPD. In conclusion, our model suggests that there is considerable variation in cost-effectiveness and, to a lesser degree, in cost-utility between the different diagnostic categories. These variations are the result of differences in survival and in quality of life with and without lung transplantation.
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PMID:Cost-effectiveness of lung transplantation in relation to type of end-stage pulmonary disease. 1519 75

The aim of this paper is to review how preexisting pulmonary diseases can be affected by altitude exposure. Obstructive (asthma and chronic obstructive pulmonary disease or COPD) and restrictive (interstitial pulmonary fibrosis), as well as pulmonary vascular diseases, will be considered, and the goal will be to provide insight and tools to clinicians to optimize the medical condition and thus the life-style of these patients. The underlying pathophysiologies and the effect of hypobaric hypoxia on these diseases will be reviewed such that techniques to assess patients will be appropriate. Therapeutic interventions, including the use of supplemental oxygen, in light of the underlying pathologic processes, will also be discussed.
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PMID:Respiratory diseases and high altitude. 1567 33

Oxidative stress is a frequent mechanism involved in the pathogenesis of bronchopulmonary disease. The cause can be exogenous, in particular related to to atmospheric pollution and tobacco smoke, or endogenous, related to mobilization of inflammatory cells (macrophages and polymorphonuclear neutrophils). In this general review, we present work demonstrating this oxidative stress and activation of inflammatory cells. We discuss the effect of oxidative stress on the bronchial tree and the need to maintain an adequate balance between oxidants and anti-oxidants. This reviews focuses on experimental studies proving the anti-oxidant effect of NAC on glutathione synthesis and on different pharmacological models. We then discuss human trials, initially experimental then in different bronchopulmonary pathologies related to oxidative stress. Acetaminophen intoxication and pulmonary fibrosis are models for use of NAC. Recent work on COPD appears to show a decrease in exacerbations, improvement in symptoms and quality-of-life, and perhaps a reduction in the alteration of ventilatory function.
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PMID:[Oxidative stress in bronchopulmonary disease: contribution of N-acetylcysteine (NAC)]. 1577 75


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